Anatomy and Physiology

Blood transports cellular requirements and products from one part of the body to another; composed of plasma (55%) and cellular component (45%) slightly alkaline (ph 7.35-7.4) 5-6 liters or 70-75 ml/kg BW (average volume) Pulmonary circulation = 1300 cc arterial (400cc) + capillary (60cc) + venous (840cc) Systemic circulation = 3000 cc arterial (550cc) + capillary (300cc) + venous (2150cc)

 Hematopoiesis blood cell production; done in the bone marrow (red), pelvis, sternum, ribs, epiphysis of long bones Erythropoiesis red blood cell production in the liver in utero (2 to 5 months old) then in bone marrow. needs iron, protein, pyridoxine (B6), cyanocobalamine (B12), folic acid, and copper Reticuloendothilial System mononuclear phagocyte system or macrophage (spleen, liver, lymphatic system, lungs)

Components

Plasma (% by weight) Proteins 7%

Albumin 58% Globulin 38%

Water 91% PLASMA (55%)

Other solutes 2%

Fibrinogen 4% Ions Nutrients Waste Products Gases Regulatory substances Neutrophils 60-70% Lymphocytes 20-25% Monocytes 3-8% Eosinophils 2-4% Basophils 0.5-1%

Formed Elements

(number per mm3)

Formed Elements (45%) Platelets 250-400 thou Leukocytes 5-9 thou Erythrocytes 2-5.8 million

Components of the Blood

Cell
Erythrocyte (RBC) Reticulocyte Leukocyte (WBC) Lymphocyte Nucleated cell Mononuclear immunocyte Large granular

Structural Characteristics
Non-nucleated cytoplasmic disk

Normal amounts
4.26.2m/mm3 0.5-2% of erythrocytes 50001000/mm3 25%-36% of leukocytes 5-10%

Function
Gas Transport

Life Span
80-120 days

Body defense mechanism Humoral and cell mediated Early response to viral infection Phagocytosis Days or years

Natural Killer cell Monocyte and Macrophage

Unknown

Large mononuclear

3-8%

Months or years

Components of the Blood

Cell
Eosinophil

Structural Characteristics
Segmented polymorphonucl ear granulocyte Segmented polymorphonucl ear granulocyte

Normal amounts
1-4%

Function
Phagocytosis, antibody mediated response against paracites, allergies Phagocytosis, early phase of inflammation

Life Span
Unknown

Neutrophil

54-67%

4 days

Basophil

Segmented Polymorphonucl ear granulocyte

0-.75%

Unknown, associated with allergies and mechanical irritation Hemostasis after vascular injury; normal coagulation and clot formation

Unknown

Platelet

Irregularly shaped cytoplasmic fragment

140k340k/mm
3

T-Cell subsets
Cell Helper T-cells Functions Secretes cytokines for immune response. Traget of HIV. These cells are also called CD4 cells Destroy virally infected cells and tumor cells, and are also implicated in transplant rejection. These cells are also known as CD8 cells Are a subset of antigen-specific T cells that persist longterm after an infection has resolved. They quickly expand to large numbers of effector T cells upon re-exposure to their cognate antigen, thus providing the immune system with "memory" against past infections. Their major role is to shut down T cell mediated immunity towards the end of an immune reaction and to suppress auto-reactive T cells that escaped the process of negative selection in the thymus. These cells can perform functions ascribed to both Th and Tc cells (i.e. cytokine production and release of cytolytic/cell killing molecules) Cytotoxic T-cells

Memory T-cells

Regulatory T-cells

Natural Killer Tcells

B-Cell subsets
Cell Plasma B-cells Functions Are large B cells that have been exposed to antigen and are producing and secreting large amounts of antibodies, which assist in the destruction of Microbes by binding to them and making them easier targets for Phagocytes and activation of the compliment system. Are specific to the antigen encountered during the primary immune response. These cells are able to live for a long time, and can respond quickly following a second exposure to the same antigen. Express IgM in greater quantities than IgG and its receptors show polyspecificity, meaning that they have low affinities for many different antigens, but have a preference for other immunoglobulins, self antigens and common bacterial polysaccharides.

Memory B-cells

B-1 Cells

Stem Cell Pool

Bone Marrow Pool

Peripheral Blood

Proliferating And maturing Storage

Storage Functional

Bone Marrow

50%

50%

Granulocyte

70% 30% Thrombocyte

0%

100%

Erythrocyte

Nutritional requirements for Erythropoiesis

Nutrient Protein Role Structural Component of plasma membranes Deficiency strength,elasticity and flexibility of membranes; Hemolytic Anemia Pernicious Anemia Megaloblastic Anemia

Intrinsic Factor Cobalamin (B12)

G.I. absorption of B12 RNA & DNA synthesis, maturation of erythrocytes RNA & DNA synthesis, maturation of erythrocytes Heme synthesis, folate metabolism Oxidative Reactions

Folate

Megaloblastic Anemia

Pyridoxine (B6) Riboflavin (B2)

Hypochromic Microcytic Anemia Normochromicnormocytic anemia

Nutritional requirements for Erythropoiesis

Nutrient Vitamin C Role Iron metabolism, maintains iron in ferrous(Fe) form Heme synthesis Respiration in mature erythrocytes Heme synthesis, possible protection against oxidative rxns in mature erythrocytes Hemoglobin Synthesis Deficiency Normochromicnormocytic anemia unknown unknown Hemolytic anemia with cell membrane fragility, lifespan or erythro in cystic fibrosis Iron Deficiency Anemia

Panthothenic Acid Niacin Vitamin E

Iron

Copper

Mobilization of tissue iron to plasma

Hypochromic-microcytic anemia

 Nursing Assessment:
Pallor conjunctiva Jaundice (hemolytic) sclera; palms of hands; soles of feet Signs of bleeding such as petechiae, ecchymosis, hematoma, epistaxis Lymph nodes enlargement Limited joint range of motion Splenomegaly or hepatomegaly

 Physical Assessment:
Auscultate heart murmur, bruits Inspect above assessment Palpate lymph nodes, location, size, bone tenderness Percuss for lung excursion, splenomegaly, hepatomegaly Evaluate joint ROM and tenderness

Normal Laboratory Values for Red Blood Cells and Platelets

Laboratory Test
Red blood cell count

Normal Value

Men

4.2 to 5.4 million/mm3

Women
Reticulocytes
Hemoglobin

3.6 to 5.0 million/mm3

1.0% to 1.5% of total RBC

Men Women

14 to 16.5 g/dL 12 to 15 g/dL

Laboratory Test
Hematocrit

Normal Value

Men Women
Mean Corpuscular

40% to 50% 37% to 47%

Volume (MCV)
Mean corpuscular

85 to 100 fL/cell

Hemoglobin concentration (MCHC)

Mean corpuscular

31 to 35 g/dL

Hemoglobin (MCH)
Platelet count

27 to 34 pg/cell 250,000 to 400,000/mm3

Normal Laboratory Values for White Blood Cells

Laboratory Test
WBC count Differential
Neutrophils Eosinophils Basophils Lymphocytes Monocytes 60 to 70% or 3000 to 7000/mm3 1-3% or 50 to 400/mm3 0.3 to 0.5% or 25 to 200/mm3 20 to 30% or 1000 to 4000/mm3 3 to 8% or 100 to 600/mm3

Value
5000 to 10,000/mm3

Diagnostic Assessment
Complete Blood Count
Red Blood Cell Count Circulating RBCs in 1 cubic mm of blood White Blood Cell Count All leukocytes present in 1 cubic mm of blood Mean Corpuscular Volume Measures the average volume or size of a single RBC Useful for classifying anemias Mean Corpuscular Hemoglobin Concentration Measures the average amount of hemoglobin by percentage in a single RBC Hematocrit Calculated as the percentage of red blood cells in the total blood volume Hemoglobin Total amount of hemoglobin

 Reticulocyte Count
Helpful in determining bone marrow function A reticulocyte is an immature RBC that still has its nucleus An elevated reticulocyte count is desirable in an anemic client or after hemorrhage Indicates the bone marrow is responding appropriately to a decrease in the total RBC mass Elevated reticulocyte count without a precipitating cause indicates polycythemia vera

Hemoglobin Electrophoresis
Detects abnormal forms of hemoglobin Such as Hemoglobin S in Sickle cell disease

 Serum ferritin, transferrin, and total iron-binding capacity (TIBC)Used to evaluate iron levels.
Ferritin measures the iron in plasma, which is also a direct reflection of total iron stores.
Transferrin is the major iron-transport protein Total Iron-Binding Capacity
The amount of iron that can be bound to serum transferrin indirectly

 Reticulocyte Count
Helpful in determining bone marrow function A reticulocyte is an immature RBC that still has its nucleus An elevated reticulocyte count is desirable in an anemic client or after hemorrhage
Indicates the bone marrow is responding appropriately to a decrease in the total RBC mass Elevated reticulocyte count without a precipitating cause indicates polycythemia vera

Hemoglobin Electrophoresis
Detects abnormal forms of hemoglobin Such as Hemoglobin S in Sickle cell disease

 Leukocyte Alkaline Phosphatase

An enzyme produced by normal mature neutrophils
Elevated levels occur during episodes of infection or stress If elevated neutrophil count without increase in LAP indicates chronic myelogenous Leukemia

Coombs test
Used for blood typing Direct test
Detects the presence of antibodies against RBCs that may be attached to a persons RBC

Indirect test
Detects the presence of circulating antiglobulins

 Capillary Fragility Test

Rumpel-Leede test Measures the vascular hemostatic function When the number of petechiae that form increases, the cause of excessive bleeding or bruising is capillary fragility rather than poor platelet action

Bleeding Time Test

Evaluates vascular and platelet activities during hemostasis Normal bleeding time ranges from 1-9 minutes

 Prothrombin Time
Measures how long blood takes to clot Reflects how much of the clotting factors II,V and X is present and how well they are functioning
Prolonged when one of the clotting factors is deficient such as
Liver disease Warfarin therapy is considered when PT is prolonged by one and a half to two times the normal value

International Normalized Ratio

Measures the same process as the PT in a slightly different way Establishing a normal mean or standard for PT Calculated by dividing the clients PT by the established standard PT

 Partial Thromboplastin Time

Platelet Agglutination/Aggregation
Ristocetin
During use of drugs such as Aspirin Anti-inflammatory agents Psychotropic drugs Platelet inhibitors Aggregation can be impaired in Von Willebrands disease

Assess the intrinsic clotting cascade and evaluates the action of factors II,V,VIII,IX,XI and XII Prolonged when any of these factors is deficient such as Hemophilia Disseminated Intravascular disease Factors II,IX and X are Vitamin K dependent and are produced in the liver Liver disease can decrease their levels and prolong PTT Heparin therapy is monitored

The ability to clump Tested by mixing the clients plasma with a substance called

 Coagulation studies
Fibrinogen normal range is 150-400 mg/dl . It is a soluble plasma protein that is decreased in DIC Fibrin degradation products (FDP): normal value is < 10 micrograms/ml. FDP is increased in fibrinolysis, thrombolytic therapy, and DIC. Fibrin D-dimer - normal is 0 - 0.5 micrograms/ml. D-dimer is the most sensitive indicator to differentiate DIC from primary fibrinolysis. It is elevated in DIC.

Radiographic Examinations
Radioisotopic Imaging
Isotopes are used to evaluate the bone marrow for sites or active blood cell formation and sites of iron storage Client is given a radioactive isotope intravenously about 3 hours before the procedure No special client preparation or follow-up care is needed

 Assess the cellularity and morphology of the bone-marrow cells. Most valuable diagnostic procedures for the diagnosis and staging of haematological disorders They can also be used to assess the cause of bone-marrow failure in patients with solid tumours Investigate pyrexia of unknown origin Examination of the aspirate or biopsy specimen may reveal infections such as tuberculosis Mycobacterium avium-intracellulare (MAI) infections histoplasmosis leishmaniasis other disseminated fungal infections. Useful in establishing the diagnosis of storage diseases such as Niemann-Pick disease Gaucher disease.

Bone Marrow Aspiration and Biopsy

 Preferred sites for bone-marrow aspiration and bone-marrow biopsy are:

1. Posterior iliac crest and anterior iliac crest (both aspiration and biopsy) 2. Sternum (aspiration only in adults)

 INDICATIONS: Unexplained anemia. Decreased presence of red blood cells, the cause of which may be a variety of reasons, the most common being deficiency of iron stores and Myelodysplasia. Thrombocytopenia. Decreased production of platelets, I.T.P. (or Idiopathic Thrombocytopenia) being most common. Physicians determine if adequate number of platelet producing cells (Megakaryocytes) are present in the bone marrow. Pancytopenia. Decreased production of all three cell lines, differentiates relative involvement of red cell, white cell and platelet lines in a disease. Leukemia, Lymphoma, or Myeloma (helpful in diagnoses, staging and determining results of the treatment) Lympho and Myeloproliferative disorders. Metastatic disease. To determine if bone marrow is involved with cancers from other sites. Chromosomal analyses Unusual infection. Such as Tuberculoses, Fungi and Fevers of Unknown Origin

 Preparation
Allay anxiety and fear Secure consent Stay with the patient during the procedure Provide emotional support by having to hold the clients hands

Procedure
Lasts from 5 to 15 minutes Amount of anesthesia or sedation depend on the physicians preference Skin over the site is cleaned with a disenfectant solution Needle is inserted in a twisting motion and the marrow is aspirated by pulling back the plunger of the syringe Pressure and several twisting motions are needed to ensure coring and loosening of a adequate amount of marrow tissue Pressure dressing or sandbags may be applied to reduce bleeding at the site

 CONTRAINDICATIONS:
Hemophilia Other Clotting disorders. (If you are taking a blood thinner your doctor needs to be informed before the procedure). Previous Radiation Therapy site.

 PRE- and POST-PROCEDURE PATIENT EDUCATION:

Procedure is performed under local anesthesia, therefore patient can eat or drink any time before or after the procedure. The patient will need to remain in a supine position (lay on the back) for about an hour after the procedure to help keep pressure on the biopsy site. The urinary bladder should be emptied before the procedure. The bandage over the biopsy site may be removed 24 hours after the procedure. Ask the patient to notify the physician of any Pain Drainage fever spreading redness around the biopsy site area. A standard consent form should be signed by all patients before the procedure.

 Follow-Up Care
cover site with a dressing after a bleeding is controlled Closely observe for 24 hours for signs of bleeding and infection A mild analgesic may be given for discomfort Ice packs can be placed over the site to limit bruising Advise client to avoid contact sports or any activity that might result in trauma to the site for 48 hours

The Bone Marrow

Sponge-like tissue found in the center of certain bonescontains stem cells that are the precursors of white blood cells red blood cells platelets. These blood cells are vital for normal body functions such as oxygen transport defense against infection Disease Clotting Blood cells have a limited lifespan and are constantly being replaced; therefore, healthy stem cells are vital

Bone Marrow Transplantation

Involves extracting bone marrow containing normal stem cells from a healthy donor, and transferring it to a recipient whose body cannot manufacture proper quantities of normal blood cells The goal of the transplant is to rebuild the recipient's blood cells and immune system hopefully cure the underlying ailment

 Precautions
Accompanied by a risk of infection Transplant rejection by the recipient's immune system Other complications Complications are exacerbated for people whose health is already seriously impaired as in late-stage cancers. Person's age or state of health may prohibit use of a bone marrow transplant. The procedure has a lower success rate the greater the recipient's age Typical cut-off age ranges from 40 to 55 years a person's general health is usually the more important factor. Are debilitating Person's ability to withstand the rigors of the transplant is a key consideration in deciding to use this treatment.

 Autologous transplant
It is typically used in cases in which a person's bone marrow is generally healthy but will be destroyed due to medical treatment for diseases such as
breast cancer and Hodgkin's disease.

Most bone marrow transplants are autologous.

Allogeneic transplants
Are more complicated Proteins called human lymphocyte antigens (HLA) that are on the surface of bone marrow cells If the donor and the recipient have very dissimilar antigens, the recipient's immune system regards the donor's bone marrow cells as invaders and launches a destructive attack against them.
Such an attack negates any benefits offered by the transplant.

 The treatment of choice for a client with leukemia Also used for lymphoma, aplastic anemia, sickle cell disease and many solid tumors Bone Marrow is the actual site of production of leukemic cells
Additional chemotherapy treatments Total body irradiation

 HLA matching is more likely if the donor and recipient are related
particularly if they are siblings

an unrelated donor may be a potential match

Only in rare cases is matching HLA types between two people not an issue

if the recipient has an identical twin

Identical twins carry the same genes and the same antigens

 Once a patient has been identified as a candidate for bone marrow transplant (BMT)
Arrangements will be made for scheduling the procedure

During this period, patients are encouraged to build up their strength with safe and suitable activities recommended by their physicians Appropriate exercise and nutrition can play an important role in preparing for and recovering from BMT

 Before undergoing BMT, patients have a series of test and procedures for screening and preparation The testing schedule is based on the patients disease process and medical history These tests and procedure may include: Blood tests to measure kidney liver heart lung hormone function Blood tests to screen for infections Bone marrow evaluation X-rays and computer-assisted tomography (CT) scans Lumbar puncture (spinal tap) Physical examination Dental examination Psychological evaluation Placement of a central venous catheter

 Pre-transplant Education
Patients and their caregivers will meet with a transplant physician and BMT nurse coordinator
To discuss the results of the testing, treatment options and the treatment plan

Touring areas where BMT patients are treated Familiarize themselves with these areas, patients and their caregivers may tour
The The The The hospital bone marrow transplant unit outpatient chemotherapy unit apheresis unit radiation oncology area

During this tour, staff from each area wil explain their role in the treatment plan Patients and caregivers are encouraged to ask questions

 Transplant Conditioning
The goal of transplant conditioning is to destroy abnormal cells or cancer cells throughout the patients body The conditioning regimen is based on the
Type of disease Previous treatment Clinical trial participation

It may consist of
Chemotherapy Radiation therapy both

Radiation may be given before the transplant as part of the conditioning regimen, or it may be given following recovery from transplant

 Transplantation
Blood and Marrow Stem Cell donation The harvest procedures for autologous or allogeneic BMT are similar The timing of the blood or bone marrow harvest depends on factors such as
The patients physical condition Donor availability Insurance approval

Peripheral blood stem cells are obtained through a process called Apheresis, which separates blood into its different components Before and during Apheresis
The donor will receive daily injections of protein growth factors to help stimulate bone marrow to make new white blood cells

 Stem cells collected by Apheresis can then be frozen for later use Donors of bone marrow typically enter the hospital the morning of the donation The donation is taken in the operating room under general anesthesia Because general anesthesia is used, donors may remain in the hospital overnight for observation Most donors bodies will replace the donated marrow within two or three weeks Healthy bone marrow may be frozen for storage and transplanted later

 Length of Stay How long the patients need to remain near the Clinic depends on a variety of factors which include:
When the transplanted marrow engrafts and begins producing healthy blood cells Patients need for red blood cell and platelet transfusions Their calorie intake The level of independence and ability to perform daily self care The level of support from caregivers at home

 Follow-up
After a patient has returned home, follow-up appointments are needed periodically These visits will take from one to four days to complete During these follow-up visits, the staff will perform tests to evaluate how well the treatment is working

Common nursing procedures of the hematologic system

Protocol for the administration of blood and blood products
1. 2. 3. 4. 5. 6. Check the agencys policy and procedure. Verify the physicians order Consent Typing and crossmatch Obtain blood intravenous access line is available. IV with normal saline Blood may not be returned to the blood bank after 20 minutes.

7. Do not keep blood or blood products in the nursing unit refrigerator. 8. Validate data of blood product with another nurse. Validate with another nurse that the clients name, ID number, blood type and Rh matches the unit of blood to be transfused. 9. Note the expiration date indicated on the blood product Observe the unit of blood for bubbles or discoloration. 10. Pre-transfusion vital signs, 15 minutes after the transfusion is initiated and immediately after the completion of the transfusion. 11. *A pre-transfusion temperature of 100 F should be reported before initiating the transfusion. Any increase in 2 degrees F in the clients temperature may be an indication of a transfusion reaction

12. *Start blood transfusion slowly (25-50 mL during the first 15 minutes). Stay with the client during first 15 min. 13. Do not administer any medications through the blood transfusion tubing. 14. Use only agency- approved blood- warming devices. 15. Blood products should not be infused longer than 4 hours 16. Discard tubings and bags used in the transfusion in a biohazard receptacle. 17. Follow protocol for transfusion reactions.

Common nursing procedures of the hematologic system

Protocol for suspected blood transfusion reaction
1. Check agency protocol. 2. Stop the infusion immediately. Change IV tubing and keep vein open with normal saline. 3. Assess the client for other signs and symptoms of transfusion reaction.

4. Notify the physician and the blood bank. 5. Send the unit of blood and tubing used to the blood bank. 6. Urine and blood samples will be required. 7. Administer prescribed drugs 8. Document the reaction and interventions.

Signs and Symptoms of Blood Transfusion Reaction

Hemolytic reaction
Chills Fever Urticaria Tachycardia chest pain or complaints of chest tightness shortness of breath dyspnea lumbar pain Nausea Rales Vomiting

hematuria,
Hypotension Wheezing

Signs and Symptoms of Blood Transfusion Reaction

Bacterial (Pyrogenic) reaction
Hypotension Abdominal pain

Fever
Chills Flushed skin

Pain in extremities
Vomiting Diarrhea

Signs and Symptoms of Blood Transfusion Reaction

Allergic reaction
Urticaria Difficulty of breathing

Pruritus
Swelling of the tongue

Pulmonary edema
Shock

Swelling of the face

Signs and Symptoms of Blood Transfusion Reaction

Circulatory Overload
Chest pain, Tightness of the chest Cough Rales Pulmonary edema Tachycardia Elevated blood pressure

Disorders of the Hematologic System

Erythrocyte Disorders
Anemia
reduction below normal level in number of erythrocytes, quantity of hemoglobin and volume of packed RBCs. Basic underlying tissue hypoxia Signs and Symptoms depends upon severity and chronicity and age.
Mild
hemoglobin 10-14 gms; asymptomatic; palpitations, dyspnea and diaphoresis following strenuous exertion.

Moderate
increased palpitations, dyspnea, and diaphoresis; fatigue at rest or during activity.

Severe
pale and exhausted all the time, sever palpitations, sensitivity to cold, loss of appetite, profound weakness, angina.

Microcytic Anemia
Iron Deficiency Anemia
Supply of iron is inadequate for optimal formation of RBCs related to:
excessive iron loss due to bleeding decreased dietary intake malabsorption

Causes:
Inadequate absorption increased requirement Inadequate intake of iron rich foods Physiologic need more in children and pregnant women Physiologic loss menstruation Blood loss trauma, GI bleeding

 Etiology and Pathophysiology

Accounts for 60% of anemias in clients over age 65. Most common cause -blood loss from gastrointestinal or genitourinary system. Normal iron excretion is less than 1 mg/day through the urine, sweat, bile, feces, and from desquamated cells of the skin 0.5 mg of iron daily or 15 mg monthly during menstruation Reduced oxygen carrying capacity of the blood, producing tissue hypoxia. Iron is stored in the body as ferritin It is formed in the intestinal mucosa, when ferritin iron joins with the protein apoferritin *ferritin is stored in the tissues, primarily in the reticuloendothelial cells of the liver, spleen, and bone marrow.

 Develops slowly through three phases:

Bodys stores of iron depleted insufficient iron is transported to the bone marrow and iron deficient erythopoiesis begins small hemoglobin deficient cells enter the peripheral circulation in large numbers

iron is needed on the hemoglobin so the oxygen molecule will attach. Adequate iron in the RBC is essential since the oxygen molecule attaches to it. An average diet supplies the body with 12 to 15 mg/day of iron, of which only 5-10% is absorbed.

 Signs and Symptoms

Palpitations Dizziness Easy fatigability Cold sensitivity Pallor Brittle nails and hair Plummer-vinsons syndrome
soreness and inflammation of mouth and tongue (stomatitis and glossitis)

 Nursing management:
Oral iron
route of choice; given after meals; liquid iron intake with straw because it stains; mixed with 1 glass cold H2O, best absorbed with Vitamin C; stool becomes tarry and constipation may occur.

Parenteral iron
avoid tissue staining by using separate aspiration injection needles; Z-tract method and deep IM; do not massage but encourage ambulation.

Dietary
increase in iron and roughage

Blood transfusion

Megaloblastic Anemia
Pernicious anemia
Vitamin B12 (cyanocobalamine) deficiency of intrinsic factor in the gastric mucosa which is necessary for absorption of Vitamin B12. Signs and Symptoms:
Hemolytic jaundice
macrolytic hypochromic

Tingling sensations, paresthesias Beefy red tongue Deficiency or absence of hydrochloric acid in the stomach

 Etiology and Pathophysiology

Inevitably develops after total gastrectomy or gastrojejunostomy. Lack of vitamin B12 alters the structure and disrupts the function of the peripheral nerves, spinal cord and brain. Lack of Vitamin B12 impairs cellular division and maturation especially in rapidly proliferating RBC's. Pernicious anemia is the bodys inability to absorb Vitamin B12 due to a lack of intrinsic factor, a substance secreted by the parietal cells of the gastric mucosa.

 Signs and Symptoms

pallor or slight jaundice complaint of weakness smooth sore beefy red tongue (glossitis) Diarrhea Paresthesias difficult prioception fair haired or prematurely gray.

 Nursing management:
Drug therapy
Vit B12 injections (monthly) for life Folic acid reverses anemia, decreases neurological symptoms

Transfusion therapy

Diagnostic assessment:
Schillings test Gastric analysis

Megaloblastic Anemia
Folic Acid Deficiency Anemia
Etiology and Pathophysiology
poor nutrition malabsorption syndrome medications that impede the absorption (oral contraceptives, anticonvulsants, methotrexate [MTX]) alcohol abuse anorexia. Lack of folic acid causes the formation of megaloblastic cells. These cells are fragile.

 Risk Factors
Pregnancy Alcoholism Hemodialysis

Signs and Symptoms

Pallor progressive weakness Fatigue shortness of breath cardiac palpitations GI symptoms are similar to B12 deficiency, but usually more severe (glossitis, cheilosis, and diarrhea);

**Neurological symptoms seen in B12 deficiency are not seen in folic acid deficiency and therefore assist in the differentiation of these two types of anemia.

 Diagnostic and Laboratory tests

Macrocytic (megaloblastic) anemia
RBC diameter > 8

MCV high with low hemoglobin Low serum folate level

Nursing Management
Energy saving techniques
e.g., shower chair, sitting to perform tasks

Monitor for dizziness, suggest position changes be made slowly. Encourage/assist with good oral hygiene before and after meals, using soft bristled toothbrush for gentle brushing of fragile gums.

 Medical Management
Oral folate, 1-5 mg/day for 3-4 months. Folate should be given along with vitamin B12 when both are deficient.

Client Education
Dietary sources of folic acid - green leafy vegetables, fish, citrus fruits, yeast, dried beans, grains, nuts, and liver. Teach clients at risk to increase their dietary intake through diet selection and supplementation. Strategies to decrease pain associated with glossitis such as eating bland and soft foods.

Aplastic Anemia
Aplastic anemia
depressed bone marrow activity secondary to:
Antineoplastics Radiation Insecticide drugs and chemical toxins.

Laboratory Assessment:
Pancytopenia Erythrocytopenia Leukocytopenia Thrombocytopenia

 Etiology and Pathophysiology

Affects all age groups and gender. Two classifications
Congenital aplastic anemia is caused by a chromosomal alteration. Acquired form may be caused by radiation, chemical agents and toxins, drugs, viral and bacterial infections, pregnancy, and idiopathic.

In about 50% of cases, the cause is unknown. There is a decrease or cessation of production of:
RBCs (anemia) WBCs (leukopenia) platelets (thrombocytopenia).

Decrease may result from damage to bone marrow stem cells, the bone marrow itself, and the replacement of bone marrow with fat. Condition may be acute or chronic.

 Clinical Manifestations
Pallor Fatigue palpitations Exertional dyspnea Infections of the skin and mucous membranes Bleeding from gums, nose, vagina or rectum Purpura (bruising) Retinal hemorrhage

Diagnostic and Laboratory Tests

Blood counts reveal pancytopenia (decreased RBC, WBC, and platelets). Decreased reticulocyte count Bone marrow examination reveals decrease in activity of the bone marrow or no cell activity.

 Therapeutic management
Identification of the cause of bone marrow suppression Bone marrow transplantation. Immunosuppression Transfusion of leukocyte-poor RBCs. Spleenectomy Blood transfusion Prevent and treat infections Drugs
Corticosteroids estrogen

Identify and withdraw offending agent

Hemolytic Anemia
Sickle Cell
hereditary, chronic form of hemolytic anemia. Etiology and pathophysiology
8% of African-Americans are heterozygous (carriers) for sickle cell anemia thereby inheriting one affected gene or the sickle cell trait 1% of African-Americans are homozygous (identical genes) for the disorder, thereby inheriting a defective gene from both parents or sickle cell anemia and are likely to experience sickle cell crisis.

 Sickle cell trait (heterozygous state) is a generally mild condition that produces few, if any manifestations.
Sickle cell anemia is caused by an autosomal genetic defect (one gene affected) that results in the synthesis of hemoglobin S. Produced by a mutation in the beta chain of the hemoglobin molecule though a substitution of the amino acid valine for glutamine in both beta chains. During decreased oxygen tension in the plasma, the hemoglobin S causes the RBCs to elongate, become rigid, and assume a crescent sickled shape. Cells clump, obstruct capillary blood flow causing ischemia and possible tissue infarction.

 Conditions likely to trigger a sickle cell

crisis include:

Hypoxia low environmental and/or body temperature excessive exercise high altitudes inadequate oxygen during anesthesia.

Other causes of sickle cell crisis include:

elevated blood viscosity/decreased plasma

volume Infection Dehydration increased hydrogen ion concentration (acidosis).

 With normal oxygenation, the sickled RBCs resume their normal shape. Repeated episodes of sickling and unsickling weaken the cell membrane, causing them to hemolyze and be removed Crisis is extremely painful and can last from 4-6 days.

Clinical manifestations
Pallor Jaundice Fatigue Irritability Priapism Large joints and surrounding tissue may become swollen during crisis

 Diagnostic and Laboratory Tests

Anemia with sickled cells noted on a peripheral smear. Hemoglobin electrophoresis to determine the presence and percentage of hemoglobin S is used for a definitive diagnosis. Elevated serum bilirubin levels. Elevated reticulocyte count.

Therapeutic Management
Bone marrow transplantation Blood transfusions Management of pain Use of chemotherapy drug hydroxyurea (Droxia) to increase hemoglobin F and decrease sickling

 Nursing Management
Teach prevention of sickle cell crisis. Referral to appropriate agency for genetic counseling and family planning. Clients who are in crisis should have the following included Management of infection
Management of pain. Administration of oxygen. Promoting hydration to decrease blood viscosity. The client in crisis should have an oral intake of at least 6 to 8 quarts per day or IV fluids of 3 liters per day.

 Monitor for complications such as:

vaso-occlusive disease (thrombosis) Hypoxia CVA renal dysfunction priapism leading to impotence, acute chest syndrome (fever, chest pain, cough, pulmonary infiltrates, and dyspnea) substance abuse.

Teach client ways to prevent sickle cell crisis

Maintaining adequate fluid intake. Clients with sickle cell disease should maintain an oral intake of at least 4 to 6 quarts per day. Avoid conditions that might predispose them to dehydration. Avoiding high altitudes Prevention of and prompt treatment of infections Stress reduction strategies Importance of regular medical follow-up.

 Avoid exposure to cold. Avoid overexertion. Adhere to vaccination schedules for pneumococcal pneumonia, haemophilus influenza type B, and hepatitis B. Importance of regular medical follow-up.

Medication Therapy
Nifedipine (Procardia) may be used for priapism. Hydroxyurea (Droxia) to increase hemoglobin F and decrease sickling Narcotic analgesics during the acute phase of sickle cell crisis. Broad spectrum antibiotics to manage acute chest syndrome. Folic acid supplements

Polycythemia Vera
An increase in the number of circulating erythrocytes and the concentration of hemoglobin in the blood Also known as polycemia, PV, or Myeloproliferative red cell disorder. Can be primary or secondary

 Etiology and Pathophysiology

Primary
Common in men of European Jewish descent. Neoplastic stem cell disorder characterized by increased production of RBCs, granulocytes, and platelets. With the over production of erythrocytes, there is increased blood viscosity resulting in congestion of blood in tissues, the liver, and spleen. Thrombi form, acidosis develops, and tissue infraction occurs as a result of the diminished circulatory flow of blood due to the increased viscosity.

 Secondary
Most common form of polycythemia vera. The disturbance is not in the development of red blood cells but in the abnormal increase of ertythropoietin, causing excessive erythropoiesis. The increase in red blood cell production due to increased erythropoietin release is a physiologic response to hypoxia. Hypoxia stimulates the release of erythropoietin in the kidney. Chronic hypoxic states may be produced by prolonged exposure to high altitudes, pulmonary diseases, hypoventilation, and smoking. The results of an increased RBC production include the increased viscosity of blood, which alters circulatory flow.

 Clinical Manifestations
Plethora
a ruddy (dark, flushed) color of the face, hands, feet, ears, and mucous membranes resulting from the engorgement or distention of blood vessels.

Symptoms associated with increased blood volume including:

Headaches Vertigo blurred vision tinnitus.

Distended superficial veins.

 Itching unrelieved by antihistamines. Symptoms associated with impaired tissue oxygenation including:
Angina Claudication Dyspnea

Erythromyalgia burning sensation of the fingers and toes. Splenomegaly in majority of those with primary polycythemia vera. Epistaxis, GI bleeding

 Diagnostic and Laboratory Tests

Elevated hemoglobin and erythrocyte count Decreased MCHC Increased WBC and basophilia Increased platelets Elevated leukocyte alkaline phosphatase Elevated uric acid Elevated cobalamin levels Increased histamine levels Bone marrow examination shows hypercellularity.

 Therapeutic Management
Management of the underlying condition (such as COPD) causing the chronic hypoxia. Repeated phlebotomy to decrease blood volume. The goal is to keep the hematocrit less than 45 to 48%. Hydration to decrease blood viscosity.

 Nursing Management
Assist in phlebotomy Measures to relieve pruritus including cool and tepid baths Accurate monitoring of fluid intake and output Nursing measures to prevent thrombotic events including:
early ambulation passive leg exercises when on bed rest avoid crossing legs maintaining adequate hydration

Administration of medications for the prevention of complications including anticoagulants.

 Medication Therapy
Myelosuppressive agents to inhibit bone marrow activity including:
hydroxyurea (Hydrea) melphalan (Alkeran) radioactive phosphorous

Allopurinol Antiplatelet agents

 Client Education
Importance of maintaining good hydration.
3 liters of fluid per day.

Facts about the disease and ways in which it can be controlled

smoking cessation.

Discuss signs and symptoms of complications associated with the disorder Prevent bleeding states Importance of a regular medical check up. Avoid products that contain iron. Ways of preventing thrombosis.

Thrombocytopenia
A decrease in the number of circulating platelets or a platelet count of less than 100,000 platelets per milliliter of blood resulting in problems of hemostasis.

 Etiology and Pathophysiology

The decrease in the number of circulating platelets may be a result of three mechanisms:
decreased production increased destruction increased consumption.

The cause of decreased production of platelets may be inherited or acquired. Increased destruction of platelets may be caused by an immune system defect.
The platelets become coated with an antibody.

 When these antibody coated platelets reach the spleen, they are recognized as foreign and are destroyed.
Platelets normally have a circulating life of 8 to 10 days but because of this immune response their life cycle is shortened. This condition is referred to as Immune Thrombocytopenic Purpura (ITP) The acute form of ITP is more common in children whereas the chronic form is more common in women between the ages of 20 to 50.

 Other causes of increased destruction of platelets include non -immune related factors such as infection or drug induced effects. A decrease in the number of functional platelets leads to bleeding disorders. Cerebral and pulmonary hemorrhage can occur when platelet counts drop below 10,000/mm3.

 Clinical Manifestations
Petechiae and purpura
anterior thorax Arms neck)

Epistaxis Gingival bleeding Menorrhagia Hematuria Gastrointestinal bleeding. Signs of internal hemorrhage

 Diagnostic and laboratory tests

Decreased hemoglobin and hematocrit if bleeding is present. Decreased platelet count. Prolonged bleeding time. Bone marrow examination to determine the etiology.
May reveal decreased platelet activity or increased megakaryocytes.

 Therapeutic management
Treatment of the underlying cause or removal of the causative agent.

Use of immunosuppressive and chemotherapeutic agents in cases of ITP

Platelet transfusions if there is active bleeding; little benefit in ITP. Splenectomy in ITP

 Nursing Management
Institute bleeding (thrombocytopenic) precautions:
Avoid intramuscular or subcutaneous injections Avoid indwelling catheters If absolutely necessary use smallest gauge needles for injections or venipunctures. Apply pressure on injection sites for 5 minutes or until bleeding stops. Discourage straining at stool, vigorous coughing and nose blowing. Avoid rectal manipulation such as rectal temperatures, suppositories, or enemas. Discourage the use of razors. Use only electric shavers. Use soft-bristled toothbrush or toothettes and avoid flossing. Pad side rails if necessary and avoid tissue trauma Avoid the use of aspirin and drugs that interfere with blood coagulation.

Monitor for signs of bleeding.

Test stools for occult blood.

Monitor CBC and platelet counts. Administer platelets as ordered. Monitor response to therapy. Steroids and immunoglobulins may be used to suppress the immune response in ITP Immunosuppressive agents may be used such as:

Medication therapy

vincristine (Oncovin)
cyclophosphamide (Cytoxan).

Platelet growth factor such as oprelvekin (Neumega).

 Client education
The client and family should be taught to monitor for signs of bleeding and when to contact the primary care provider. Instructions on bleeding precautions including:
use of soft-bristled toothbrush avoidance of flossing

prevention of tissue trauma and injury including vigorous sexual intercourse

using an electric razor for shaving.

 Teach client to avoid drugs that contain aspirin and others that interfere with coagulation. Discuss medication dosing, schedule, and side effects. Teach the importance of regular medical follow up and platelet monitoring.

Hemophilia
A group of hereditary clotting factor disorders characterized by prolonged coagulation time that results in prolonged and sometimes excessive bleeding.

 Etiology and Pathophysiology

Hemophilia A and B are X-linked recessive disorders transmitted by female carriers, displayed almost exclusively in males. Hemophilia A (classic hemophilia) is a deficiency in Factor VIII . Most common form of hemophilia. Hemophilia B (Christmas disease) is a deficiency in Factor IX. Hemophilia A & B are clinically identical. In clients with hemophilia A &B, platelet plugs are formed at the site of bleeding, but the clotting factor impairs the coagulation response and the capacity to form a stable clot.

 Von Willebrands Disease

a related disorder caused by a deficiency of the von Willebrands factor (vWF), which is necessary for factor VIII activity and platelet adhesion. This disorder affects men and women equally

 Clinical Manifestations
Persistent and prolonged bleeding from small cuts and injuries. Delay of onset of bleeding after an injury. Subcutaneous ecchymosis and subcutaneous hematomas. Gingival bleeding Gastrointestinal bleeding. Hematuria Pain, paresthesias, or paralysis resulting from nerve compression of the hematomas. Hemarthrosis

 Diagnostic and laboratory tests

Specific factor assays to determine the type of hemophilia present APTT is increased in all types of hemophilia Bleeding time is prolonged in von Willebrand disease Decreased factor VIII in hemophilia A, vWF disease, and factor IX in hemophilia B.

 Therapeutic management
Treatment is the replacement of the deficient coagulation factor(s).

Hemophilia A
cryoprecipitate containing 8-100 units of factor VIII per bag at 12- hour intervals until bleeding ceases. Freeze-dried concentrate of Factor VIII may also be given.

Hemophilia B:
plasma or factor IX concentrate given Q 24 hours or until bleeding ceases.

 Von Willebrands disease

cryoprecipitate containing 8-100 units of factor VIII per bag at 12-hour intervals until bleeding ceases. Desmopressin (DDAVP) given intravenously may also be used.

Supportive treatment for hemarthrosis including arthrocentesis and physiotherapy. Control of topical bleeding with hemostatic agents, pressure, and application of ice. Management of complications associated with hemorrhage.

 Nursing Management
Teach the client and family about the disease and therapeutic regimen Refer for genetic counseling and family planning. Refer to the National Hemophilia Foundation for support and counseling Monitor for signs of complications including hemarthrosis and intracranial bleeding Assist in the management of pain associated with hemarthrosis Control bleeding and maintain hemostasis Administer medications as prescribed.

 Client Education
Signs and symptoms requiring immediate medical attention Precautions to prevent bleeding. Medic Alert bracelet indicating about the hemophilia. Maintain good dental hygiene to decrease the necessity of invasive dental procedures. Adhering to scheduled visits and follow up care

Disseminated Intravascular Coagulation

Also known as Consumption coagulopathy a syndrome characterized by abnormal initiation and acceleration of clotting and simultaneous hemorrhage. The paradoxical bleeding that occurs is a result of the consumption of clotting factors and platelets. The syndrome is usually precipitated by an underlying pathologic condition.

 Etiology and Pathophysiology

Mortality rate associated with DIC is as high as 80% with the most frequent sequela being hemorrhage. The syndrome is precipitated by conditions such as widespread tissue damage, hemolysis, hypotension, hypoxia, and metabolic acidosis. The underlying condition causes initiation and widespread formation of clots in the vascular system either through the activation of factor XII, factors II and X, or the release of tissue thromboplastin. Substances necessary for clotting are used at a more rapid rate than it can be replaced.

 As the clotting continues, the fibrinolytic pathway is activated to dissolve the clots formed. Clotting factors become depleted while fibrinolysis continues. Platelets decrease, clotting factors II, V, VIII, and fibrinogen are depleted Fibrin degradation products (FDP) are released as a result of fibrinolysis. Fibrin degradation products (FDP), which are potent anticoagulants used to lyse the clots further increases the bleeding state. With the depletion of clotting factors and the increase in fibrin degradation products stable blood clots no longer form and hemorrhage occurs.

 Risk factors
Venomous snake bite Sepsis Trauma Obstetric complications Neoplasms Vascular disorders Hypoxia Drug reactions Liver disease Acute hemolysis Extensive burns Prosthetic devices

 Clinical Manifestations
Integumentary
Decreased skin temperature Pallor Purpura Ecchymoses Hematomas Acral cyanosis Superficial gangrene Altered sensation Gingival bleeding Bleeding from puncture sites

 Gastrointestinal
Hemoptysis Melena Occult blood in stool or vomitus Abdominal distention Abdominal pain

Respiratory
Dyspnea Orthopnea Tachypnea Decreased breath souns Chest pain

 Genitourinary
Hematuria Oliguria

Nervous system
Vision changes Dizziness Headache Irritability Anxiety Confusion Seizures

 Cardiovascular
Decreased pulses Decreased capillary filling time Tachycardia Venous distention

Musculoskeletal
Joint pain Bone pain Weakness

 Diagnostic and laboratory tests

Prothrombin time - prolonged
Partial thromboplastin time prolonged Thrombin time prolonged Fibrinogen decreased Platelets- decreased Fibrin split (degradation) products elevated

Factor assays (factors V, VII, VIII, X, XIII)reduced

D-dimers- elevated

 Therapeutic Management The priority of therapeutic management is to initiate treatment of the underlying medical condition that precipitated DIC. Life-threatening hemorrhage accomplished by administering specific blood components based on the identified deficiency:
Platelets for thrombocytopenia cryoprecipitate to replace fibrinogen, and factors V and VII fresh frozen plasma to replace all clotting factors except platelets.

Use of Heparin or Antithrombin III (AT-III) to control intravascular clotting. Their use is controversial.

 Nursing Management
Assess client carefully for evidence of bleeding and altered tissue oxygenation Institute thrombocytopenic precautions Monitor intake and output hourly. Administer blood products as indicated. Monitor for signs of complications such as:
renal failure pulmonary embolism cerebrovascular accident acute respiratory distress syndrome

Monitor effectiveness of therapy and pharmacologic interventions. Provide emotional support to client and family.

 Medication therapy
Heparin Antithrombin III Epsilon aminocaproic acid (Amicar)
to inhibit fibrinolysis

Blood products
FFP Platelets cryoprecipitate

 Client education
Regarding the syndrome and explain treatments and interventions

To report symptoms of complications including:

abdominal pain Headache visual disturbances

pain

Thrombocytopenic precautions

Neutropenia
Refers to a decrease (less than 2000/mm3) in the neutrophil count either as a result of decreased production or increased destruction. The neutrophil plays a major role in

phagocytosis of disease-producing microorganisms. Consequently, a decrease in their numbers increases the individuals risk for infection.

 Etiology and Pathophysiology

Neutropenia is not a disease but a syndrome. May occur as a primary hematologic disorder but may also be caused by drugs, autoimmune disorders, infections, and other medical conditions such as severe sepsis and nutritional deficiencies. If the leukocyte count is decreased, or if immature white blood cells predominate in the circulation, the normal phagocytic function of these cells is impaired. Neutrophils constitute about 70% of the total circulating white blood cells. Normally, the neutrophil count is above 2000/mm3.

 Signs and Symptoms

There are no real symptoms associated with neutropenia.

Diagnostic and laboratory tests

Neutrophil count less than 1000 to 1500. Bone marrow examination to examine cell morphology helps distinguish the etiologic factor causing the neutropenia.

 Therapeutic management
If the etiology of neutropenia is druginduced, discontinuation of the medication is indicated. Corticosteroids are used if the etiology is immunologic If the etiology is decreased production, growth factors (granulocyte/macrophage colony stimulating factor or GM-CSF) may be used.

If client develops a fever, identification and treatment of the infection is instituted

 Nursing Management
Monitor for signs of infection. Monitor temperature elevations Obtain cultures suspected as sites of infection. Administer antibiotics as prescribed and evaluate their effectiveness. Administer medications that stimulate the production of neutrophils. Enforce strict hand washing by all individuals in contact with the client. Institute reverse isolation. Use private room with HEPA filtration if possible. Avoid invasive procedures. Fresh flowers and fruits should not be permitted in the clients room.

 Client education
Teach client and family to report signs of fever Teach the client and individuals who come in contact with the client about strict hand washing and reverse isolation procedure. Teach client methods to maintain good personal hygiene. Explain to the client and family about the condition and the rationale of therapeutic interventions.

Leukemia
A malignant disorder of the bloodforming tissues of the bone marrow, spleen, and lymph system characterized by unregulated proliferation of WBCs and their precursors.

 Etiology and Pathophysiology

Basis of the classification of the different types of leukemia
The type of WBC affected (granulocyte, lymphocyte, monocyte)
The duration of the disease (acute or chronic)

If the majority of the leukemia cells are primitive, the leukemia is classified as acute; if the leukemic cells are mostly mature (well differentiated), the leukemia is classified as chronic.

 Classifications
Acute lymphocytic/lymphoblastic leukemia (ALL)
Peak incidence at 2 to 4 years of age. Immature granulocytes proliferate and accumulate in the marrow

Chronic lymphocytic leukemia (CLL)

More common in men and mainly between the ages of 50 and 70. Abnormal and incompetent lymphocytes proliferate and accumulate in the lymph nodes and spreads to other lymphatic tissues and the spleen. Most of the circulating cells are mature lymphocytes.

 Acute myelogenous/myelocytic leukemia (AML)

All age groups are affected with a peak incidence at age 60. There is uncontrolled proliferation of myeloblasts, which are the precursors of granulocytes. They accumulate in the bone marrow.

Chronic myelogenous leukemia (CML)

Uncommon in people under 20 years of age. The incidence rises with age. - There is uncontrolled proliferation of granulocyte resulting in increased circulating blast (immature) cells.

 The marrow expands into long bones because of this proliferation and also extends into the liver and spleen. - In most cases the Philadelphia chromosome, a characteristic chromosomal abnormality, is present. Abnormal cells can continue to multiply, infiltrate, and damage the bone marrow, spleen, lymph nodes, liver, kidneys, lungs, gonads, skin, and central nervous system (CNS) The bone marrow becomes functionally incompetent with resulting bone marrow suppression

Acute leukemia
has a rapid onset, progresses rapidly, with a short clinical course If left untreated, death will result in days or months.

The symptoms of acute leukemia relate to a depressed bone marrow, infiltration of leukemic cells into other organ systems, and hypermetabolism of leukemia cells.

Chronic leukemia
has a more insidious onset with a more prolonged clinical course. Clients with the chronic form of leukemia are usually asymptomatic early in the disease. The life expectancy may be more than five years. Symptoms of chronic leukemia relate to hypermetabolism of leukemia cells infiltrating other organ systems. The cells in this type of leukemia are more mature and function more effectively.

 Clinical Manifestations
Fever Night sweats Bleeding Ecchymoses Lymphadenopathy Weakness Fatigue Shortness of breath Decreased activity tolerance Bone or joint pain Visual disturbances

Gingival bleeding
Epistaxis Pallor Splenomegaly hepatomegaly

Pruritic vesicular lesions

Anorexia Weight loss

 Diagnostic and Laboratory Tests

Increased WBC (in CLL and CML) A normal, decreased or increased WBC (in ALL and AML). Decreased reaction to skin sensitivity tests (anergy). Bone marrow tests reveal excessive blast cells in AML Philadelphia chromosome found in 90% to 95% in clients with CML. BCR/ABL gene is present in virtually all clients with CML. Bone marrow biopsy and aspirate is the definitive diagnostic test.

 Therapeutic management
Induction of remission with chemotherapy and radiation therapy.

Bone marrow and stem cell transplantation.

Nursing Management
Provide for diversionary activities. Maintain good nutrition. Enlist the assistance of a dietician in maximizing and meeting the nutritional needs of the client.

Assist the client in maintaining good personal hygiene. Measures to promote oral hygiene should be instituted.

 Refer client and family to appropriate agencies such as Meals on Wheels, American Cancer Society, and the Leukemia Society.
Provide emotional support to the client and family. Refer to appropriate agency, organization, or professional for counseling and support. Administer drugs that are prescribed and monitor for side effects. - Monitor laboratory results to evaluate effectiveness of interventions and therapy Prepare the client for bone marrow transplantation if this is included in the treatment plan. Review and institute the care of a client receiving chemotherapy

 Review and implement the nursing care of a client undergoing radiation therapy
Assist in bone marrow biopsy. Apply pressure on the site for five minutes or until bleeding stops. Frequently assess the site for signs of bleeding up to four hours after the procedure. Plan activities to prevent fatigue. Provide measures for uninterrupted rest and sleep.

Institute neutropenic and bleeding precautions

 Medication Therapy
alkylating agents
Busulfan [Myleran] Anthracyclines Doxorubicin [Adriamycin]

Antimetabolites
Fludarabine [Fludara]

corticosteroid
Prednisone

plant alkaloids
Vincristine [Oncovin]

Malignant Lymphomas
Lymphoma is a group of malignant neoplasms that affects the lymphatic system resulting in the proliferation of lymphocytes.
Lymphomas can be classified as:
Hodgkins diease Non-Hodgkins lymphoma

Hodgkins Disease
Etiology and Pathophysiology
More common in men and has two peaks ; 15-35 years of age and 55 to 75 years of age. Incidence is higher in whites than in African Americans Cause unknown Several factors have been identified to contribute:
infection with the Epstein-Bar virus (EBV) familial pattern exposure to toxins

Characterized by the presence of Reed-Sternberg cell The tumor originates in a lymph node (in majority of cases from the cervical nodes) and infiltrates the spleen, lungs, and liver

 Clinical Manifestations
Usually begins with a firm and painless enlargement of one or more lymph nodes on one side of the neck. Fatigue Weakness Anorexia Dysphagia Dyspnea Cough Jaundice Abdominal pain Bone pain PruritusDevelopment of severe but brief pain at the site of Hodgkins after ingestion of alcohol.

 B Symptoms
Fever without chills Night sweats Unintentional 10% weight loss Enlarged lymph nodes Splenomegaly Hepatomegaly

 Diagnostic and Laboratory Tests

Normocytic, normochromic anemia Neutrophilia, monocytophilia, and lymphopenia Presence of Reed-Strenberg cells in excisional bone biopsy Mediastinal lymphadenopathy revelaed by chest x-ray, CT scan, and radioisotope studies. Mediastinal mass and pulmonary infiltrates may be seen on chest x-ray Absent or decreased response to skin sensitivity testing known as anergy.

 Therapeutic Management

Lymphangiography is used to evaluate abdominal nodes. Staging laparotomy is performed to obtain specimen of retroperitoneal lymph nodes and to remove the spleen. Stage I indicates involvement of a single lymph node region Stage IV indicates diffuse or disseminated involvement of 1 + extralymphatic organs, with or without lymph node involvement (liver, lung, marrow, skin). Radiation therapy for stages IA, IB. IIA, and IIB. Combination chemotherapy for stages III, IV and all B stages. Combination radiation and chemotherapy for stages IA and IB.

 Nursing Management
Institute nursing interventions for clients on chemotherapy or radiation therapy. Assist in balancing activity with periods of rest Provide and assist in maintaining good nutritional state Provide measures to diminish the discomfort associated with pruritus Provide interventions to enable client to deal with body image changes such as alopecia, weight loss, and sterility. Refer client and family to appropriate agencies for support. Plan interventions for the prevention of infection.

 Medication therapy
Chemotherapeutic agents Biologic therapy agents

Client Education
nature of the disease, the course of therapy and associated interventions. medications prescribed, preacautions, and side effects. symptoms necessitating immediate medical intervention such as the occurrence of bleeding, infection, or fever

Non-Hodgkin Lymphoma
Etiology and Pathophysiology
Most common form of lymphoma. Affects usually adults from 50 to 70 years old. More common in men than women and in whites No known cause but is linked to viral infections, immune disorders, genetic abnormalities, exposure to chemicals, and infection with Helicobacter pylori

 Has a similar pathophysiology to Hodgkins disease although Reed-Sternberg cells are absent and the method of lymph node infiltration is different. In majority of cases the disease involves malignant B cells Lymphoma usually originates outside the lymph nodes. The lymphoid tissues involved become infiltrated with malignant cells. The cells that make up the lymphoid tissue become abnormal and crowd out normal cells.

 Clinical Manifestations
Oainless lymoh node enlargement B symptoms Hematuria Nausea Vomiting Abdominal pain Peripheral neuropathy Cranial nerve palsies Headaches Visual disturbances Changes in mental status and seizures Shortness of breath, cough and chest pain

 Diagnostic and Laboratory Tests

Lymphocytopenia X-ray may reveal pulmonary infiltrates. Lymph node biopsy helps to identify the cell type and pattern

Therapeutic Management
Staging of the disease is undertaken. This is based on data obtained from CT scans and bone marrow bipsies Combination chemotherapy

 Radiation alone or in combination with chemotherapy for stage I and II. Biologic therapy with alpha interferon, interleukin-2, and tumor necrosis factor. Administration of rituximab (Rituxan), a monoclonqal antibody against the CD20 of malignant B lymphocytes, which causes cell lysis and death

Nursing Management and Client Education same as Hodgkins disease.