HEMATOLOGI C and PERIPHERAL VASCULAR SYSTEM

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Blood

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Exchange medium between the external environment and the bodys cells Fluid tissue that transports nutrients, wastes and body heat within the body through

Functions of the Blood

Transporting oxygen, nutrients, hormones, and metabolic wastes Protecting against invasion of pathogens Maintaining blood coagulation Regulating fluids, electrolytes, acids, bases, 6/14/12

Components of Blood
Cellular component Fluid component

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Cellular Component

Erythrocytes Leukocytes Thrombocytes

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Erythrocytes

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Erythrocytes

Biconcave Anucleated Contains hemoglobin Carries O2 from the lungs to the tissues and CO2 back from the tissues to the lungs Most common type of blood cell Hematocrit 6/14/12

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Erythrocytes

120 day Women: 4-5 M/ L Men: 4.5- 6 M/ L

Anemia Polycythemia

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Characteristics

Size

Normocytic Microcytic Macrocytic Normochromic Hypochromic

Color

Shape

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RBC Production and Regulation

Eryhtopoiesis- RBC production Stimulated by TISSUE HYPOXIA

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RBC Destruction

Hemolysis- destrcution of RBC Destroyed by phagocytes in the spleen, liver, marrow and lymph nodes

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Leukocytes

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Leukocytes

Part of the bodys defense against microoranism Diapedesis Chemotaxis 5,000-10,000 cells/L

Leukocytosis Leukopenia

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Leukocytes

2 major groups Granulocytes Agranulocytes

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Granulocytes

Horse-shoe shaped nuclei and contain large granules in the cytoplasm Mature fully in the bone marrow before being released in the blood stream 3 kinds

Netrophils Eosinophils Basophils

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Neutrophils
Comprise of 60% -70% of circulating WBC First cells to arrive at the site of injury? Their number increase during inflammation or infection LS of 10 hours 6/14/12

Eosinophils

Comprise of 1% to 3% of circulating WBC Large number in the mucosa and intestines Numbers increase during allergic reaction and parasitic infections

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Basophils

Comprise less than 1% Contain heparin, histamine, and other inflammatory mediators Increase in number during allergic and inflammatory reaction

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Agranulocytes

Includes monocytes and lymphocytes that enters the blood stream before final maturation

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Monocytes

Largest of the WBCs Phagocytic cells that mature into macrophages Dispose of foreign and waste material esp during inflammation Active part of the immune system

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Lymphocyte

Mature in the lymphoid tissue into :

B cell-involve in humoral immune response and antibody production T cell- involves in the cell mediated process
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Thrombocytes

Thrombocytosis 6/14/12 thrombocytopenia

Cell fragments that have no nucleus and cannot replicate Metabolically active and produce ATP and produce mediators required for clotting The number of circulating platelets control thrombopoietin release LS- 10 days 250,000- 400,000 L

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Vessel spasm Formation of platelet plug Development of insoluble fibrin Clot retraction Clot resolution 6/14/12

Series of complex interactions between platelet and clotting mechanisms that maintains steady state of blood volume, blood flow and BP on the injured blood vessel Stages

Hemostasis (Blood Clotting)

Clotting Factors

Extrinsic Factors

Factor I- Fibrinogen Factor II- Prothrombin Factor III- Thromboplastin Factor V- Proaccelerin Factor VII- Serum Prothrombin conversion Acceleration Factor X- Stuart Prower Factor

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Clotting Factors

Intrinsic factors

Factor VIII- antihemophilic globulin Factor IX- plasma thromboplastin component (Chrsitmas factor) Factor XI- plasam thromboplastin antecedent Factor XII- Hageman Factor FactorXIII- fibrin stabilizing factor Factor IV- Calcium

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Vessel spasm Caused by THROMBOXANE w/c is released by platelet during blood vessel damage This will cause vasoconstriction for 6/14/12 about 1 minute w/c

Platelet Plug Formation

Platelets adhere to injured vessel lining* Anchored platelets release chemicals that attract more platelets to the site As more and more platelets pile up a small mass called platelet plug or white thrombus is formed 6/14/12

Development of Insoluble Fibrin

Fibrin produced by the activation of both extrinsic and intrinsic clotting pathway Intrinsic- activated when blood contacts collagen in the injured vessel - slower Extrinsic- activated when blood is exposed to tissues 6/14/12 - faster

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Clot Retraction

After the clot is stabilized (after about 30 mins), trapped platelets contract Fibrin strands pull the broken portion of the vessel closer together Growth factor is released by platelets to stimulate cell division and tissue repair 6/14/12

Clot Dissolution
Fibrinolysis Begins after the clot has formed, restoring blood flow and promoting tissue repair Plasminogen is converted to plasmin which dissolves fibrin clot and 6/14/12 other coagulation factors

Fluid Component (plasma)

Water Lipids Inorganic salts Gases Waste products Proteins

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Plasma Proteins

Albumin Gamma globulin

IgG, IgA, IgM, IgE, IgD

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LABORATORY TESTS

Complete blood count Reticulocyte count Hemoglobin erythropoiesis Leukocyte alkaline phosphatase Coombs test Serum ferritin, transferrin, and total iron binding capacity

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Red Blood Cell Count

Value

FemaleMale-

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ANEMIA

A condition in which the hemoglobin concentration or the circulating RBC is lower than

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Causes

1. Loss of RBC- occurs with bleeding 2. Decreased RBC production 3. Increased RBC destruction

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General Manifestations
Pallor Weakness, fatigue Increase heart and respiratory rate Angina, dyspnea Bone pain Headache, dizziness, dim 6/14/12 vision

Types of Anemia by Cause

Blood loss anemia Nutritional anemia

Hemolytic anemia

Iron deficiency anemia Vit B12 deficiency anemia Folic acid deficiency anemia Sickle cell anemia Thalassemia G6PD anemia

Aplastic anemia
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A. Blood Loss Anemia

Results from acute or chronic bleeding RBC and other blood components are loss Circulating blood volume decreases Decresed CO Compensatory mechanisms activated
Tachycardia 6/14/12

and tachypnea

B. Nutritional anemia

Iron deficiency anemia Vit B12 deficiency anemia Folic acid deficiency anemia

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IRON DEFICIENCY ANEMIA

Most common type When iron supply is inadequate for optimal RBC formation Body cannot synthesize Hgb w/o Iron Most of the iron are reused but some are continuously excrete in the feces RBC become microcytic, hypochromic and malformed 6/14/12

Causes of IDA

Dietary deficiencies

Vegetarian diet Inadequate protein intake Gastrectomy Chronic diarrhea Malabsorption syndome Pregncy and lactation GI bleeding

Decreased absorption

Increased metabolic requirements

Blood loss
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Iron Deficiency Anemia

Laboratory findings 1. CBC- Low levels of Hct, Hgb and RBC count 2. low serum iron, low ferritin 3. Bone marrow aspiration- MOST definitive
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Manifestations of IDA

General manifestations Spoon shaped nails (Koilonychia) Cheilosis-dry cracked lips Smooth sore tongue Pica

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Iron Deficiency Anemia

Medical management 1. Hematinics 2. Blood transfusion

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Iron Deficiency Anemia

Nursing Management 1. Provide iron rich-foods

Organ meats (liver) Beans Leafy green vegetables Raisins and molasses

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Iron Deficiency Anemia

Nursing Management 2. Administer iron Oral preparations tabletsFe fumarate, sulfate and gluconate Advise to take iron ONE hour before meals Take it with vitamin C Continue taking it for several months 6/14/12

Iron Deficiency Anemia

Nursing Management 2. Administer iron Oral preparations- liquid It stains teeth Drink it with a straw Stool may turn blackish- dark in color Advise to eat high-fiber diet to counteract constipation
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Iron Deficiency Anemia

Nursing Management 2. Administer iron IM preparation Administer DEEP IM using the Z-track method Avoid vigorous rubbing Can cause local pain and staining
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Vit B12 Deficiency Anemia

Vit B 12- necessary for DNA synthesis and neurologic function Deficiency may result to impaired cell division and maturation of cell nucleus CAUSES

Inadequate intake Poor GI absorption 6/14/12

Vit B12 Deficiency Anemia

Pernicious anemia- failure to absorb dietary Vit B12 Due to lack of intrinsic factor as a result of gastritis or resection Characterized by macrocytic ,misshapen with thin membrane 6/14/12

Manifestations

Pallor Slight weakness and jaundice Diarrhea Smooth,sore,red beefy tongue Paresthesia Problems with proprioception

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Vit B12 Deficiency Anemia

Laboratory findings 1. Peripheral blood smearshows giant RBCs, WBCs with giant hypersegmented nuclei 2. Very high MCV 3. Schillings test 4. Intrinsic factor antibody test

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Vit B12 Deficiency Anemia

Medical Management 1. Vitamin supplementation

Folic acid 1 mg daily Vegetarians should have vitamin intake

2. Diet supplementation

3. Lifetime monthly injection of IM Vit B12

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Vit B12 Deficiency Anemia

Nursing Management 1. Monitor patient 2. Provide assistance in ambulation 3. Oral care for tongue sore 4. Explain the need for lifetime IM injection of vit B12

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Folic Acid Deficiency Anemia

Folic Acid- needed fro DNA synthesis and normal maturation of RBC SOURCES

Green leafy veggies, fruits,cereals meat

Characterized by fragile megaloblastic (large but immature)cells 6/14/12

Folic Acid Deficiency Anemia

Causative factors 1. Alcoholism 2. Mal-absorption 3. Diet deficient in uncooked vegetables 4. increased dietary demand

Pregnant mothers

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Folic Acid Deficiency Anemia

Pathophysiology Decreased folic acid impaired DNA synthesis in the bone marrow impaired RBC development, impaired nuclear maturation but CYTOplasmic maturation continues large size 6/14/12

Folic Acid Deficiency Anemia

Manifestations Gen signs No neurologic effect Similar to Vit b12 anemiaglossitis,cheilosis diarrhea Maternal folic acid deficiencyneural tube defect like myolomeningocele
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D. HEMOLYTIC ANEMIA

Characterized by premature destruction(lysis) of the RBC -> hematopoietic activity of the bone marrow increases -> increased reticulocytes in the blood stream Characterized by normocytic and normochromic RBC

Sickle cell Thalassemia 6/14/12

Causes of Hemolytic

Intrinsic

RBC cell membrane destruction HGB structure defect Inherited enzyme defect

Extrinsic
Drugs, chemicals Toxins and venoms Bacterial and other infection Trauma Burns 6/14/12

Sickle Cell Anemia

Hereditary chronic hemolytic anemia Characterized by episodes of sickling (RBC become crescent shape) Transmitted as an autosomal recessive genetic defect Causes synthesis of abnormal form of hemoglobin Shorten life span most commonly due to infection
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Factors that precipitate sickling

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Hypoxia Cold Vasoconstriction Excessive exercise Anesthesia Dehydration inc Hgb Infection Acidosis-reduces affinity of Hgb to O2

PATHOPHYSIOLOGY

Abnormal substitution of a single amino acid VALINE for GLUTAMIC ACIC in the beta chain of Hgb C A C G T G HbS

C T C G A G HbA

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PATHOPHYSIOLOGY

Factors cause defective hemoglobin to acquire a rigid, crystal-like Cshaped configuration Sickled RBCs will adhere to endothelium pile up and plug the vessels ischemia results pain, 6/14/12 swelling and fever

MANIFESTATION
pallor Fatigue Jaundice Irritability Spleenomealy, hepatomegaly Renal insuffiency 6/14/12 Gall stones

COMPLICATIONS

Vasooclusive or thrombotic crisis

Occurs when sickling develop in microcirculation

Painful and last an average of 4-6 days Priapism Abdominal pain Stroke Skin ulcers 6/14/12 Aplastic anemia

Sickle Cell Anemia

SICKLE CELL CRISISsevere episodes of fever of intense pain that are the hallmark of this disorder

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Medical Management 1. Bone marrow transplant 2. Hydroxyurea

Hemolytic Anemia: Sickle Cell

Increases the HbF

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3. Long term RBC trnasfusion

Nursing Management 1. manage the pain

Hemolytic Anemia: Sickle Cell

Support and elevate acutely inflamed joint Relaxation techniques analgesics

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Nursing Management 2. Prevent and manage infection

Monitor status of patient Initiate prompt antibiotic therapy

Hemolytic Anemia: Sickle Cell

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Hemolytic Anemia: Sickle Cell Nursing Management 3. Promote coping skills Provide accurate information Allow patient to verbalize her concerns about medication, prognosis and future pregnancy
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Hemolytic Anemia: Sickle Cell Nursing Management 4. Monitor and prevent potential complications Provide always adequate hydration Avoid cold, temperature that may cause vasoconstriction
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Nursing Management 4. Monitor and prevent potential complications

Hemolytic Anemia: Sickle Cell

Leg ulcer
Aseptic

technique

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Hemolytic Anemia: Sickle Cell Nursing Management 4. Monitor and prevent potential complications Priapism
Sudden

painful erection Instruct patient to empty bladder, then take a warm bath
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THALASSEMIA

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Inherited disorder of hemoglobin synthesis in which either the alpha or beta chains of the hemoglobin molecule are missing or defective Leading to deficient hemoglobin production

THALASSEMIA

Alpha-thalassemia (Cooleys anemia)

Mediterranean anemia (southern Italy, Greece)

Beta Thalassemia ( Thalasemia minor)

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People with Asian ancestry (Thailand, Philippines, China) Mild maifestations

Manifestations

Thalassemia minor

Asymptomatic Mild- mod anemia mild splenomegaly Bronze skin coloring Bone marrow hyperplasia

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Manifestations

Thalassemia major

Severe anemia Heart failure Liver and spleen enlargement Bone marrow exapnsion Enlarged skull and facial bones Jaundices Accumulation of iron in the diff organs

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Management

Blood transfuision Fatal in the first few years of life Reproductive age- hereditary

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Hereditary defect in RBC metabolism Defective gene is located on the X-chromosome G6PD-enzyme that catalyzes glycolysis-> the process in which an RBC derives cellular energy 6/14/12

GLUCOSE-6-PHOSPHATE DEHYDROGENASE ANEMIA

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Defect in G6PD- causes direct oxidation of hemoglobin damaging the RBC Hemolysis occurs when exposed to stressors (aspirin, sulfonamides,vitk derivatives) inc metabolic

Manifestations

Pallor Jaundice Hemoglobinuria

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POLYCYTHEMIA VERA

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Refers to an INCREASE volume of RBCs The hematocrit is ELEVATED to more than 55% Clasified as Primary or Secondary or relative

POLYCYTHEMIA VERA

Primary Polycythemia
A proliferative disorder in which the myeloid stem cells become uncontrolled uncommon 6/14/12

Primary Polycythemia

Manifestations
Hypetension Venous stasis Thrombosis hemorrhage

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Secondary Polycythemia

Most common form Eryhtropoietin level is elevated May affect person at any age or race Usually develops in response to hypoxia MANIFESTATIONS

Similar to thos with primary

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Relative Polycythemia

Occurs due to the fluid deficit not excess RBC Total RBC is normal Increased hematocrit because of increased cell concentration Rehydration

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Polycythemia

POLYCYTHEMIA VERA Causative factor

unknown

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Polycythemia

POLYCYTHEMIA VERA Pathophysiology

The stem cells grow uncontrollably The bone marrow becomes HYPERcellular and all the blood cells 6/14/12 are increased in number

Polycythemia

POLYCYTHEMIA VERA Pathophysiology

The spleen resumes its function of hematopoiesis and enlarges 6/14/12 Blood becomes thick and

Polycythemia

POLYCYTHEMIA VERA Pathophysiology

Overtime, the bone marrow becomes fibrotic

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POLYCYTHEMIA VERA

Assessment findings
1. hpn 2. Splenomegaly 3. headache,tinnitus 4. dizziness, blurred vision 5. Angina, dyspnea and thrombophlebitis Intemittent claudication 6/14/12

Polycythemia

POLYCYTHEMIA VERA Laboratory findings

1. CBC- shows elevated RBC mass 2. Normal oxygen saturation 3 6/14/12 Elevated WBC and

Polycythemia

POLYCYTHEMIA VERA Complications

1. Increased risk for thrombophlebitis, CVA and MI 2. Bleeding due to dysfunctional blood cells 6/14/12

Polycythemia

POLYCYTHEMIA VERA Medical Management

1. To reduce the high blood cell massPHLEBOTOMY 2. Allopurinol 3. Dipyridamole 6/14/12 4. Chemotherapy to

APLASTIC ANEMIA

A condition characterized by decreased number of RBC as well as WBC and platelets

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causes
1. Environmental toxinspesticides, benzene 2. Certain drugsChemotherapeutic agents, chloramphenicol, phenothiazines, Sulfonamides 3. Heavy metals 4. Radiation 6/14/12 Viral nfection 5.

Pathophysiology

Toxins cause a direct bone marrow depression acellualr bone marrow decreased production of blood elements

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Manifestations

Pancytopenia 1. fatigue 2. pallor 3. dyspnea 4. bruising 5. splenomegaly 6. retinal hemorrhages 7. infection

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DISORDERS OF THE WBC

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LEUKEMIA

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Cancer of the blood or bone marrow characterized by an abnormal white blood cells Leukemia cells dont die when they should

Leukemia

The leukemias are named after the specific lines of blood cells afffected primarily

Myeloid Lymphoid

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Leukemia

The leukemias are named also according to the maturation of cells ACUTE

The cells are primarily immature

CHRONIC
The cells are primarily 6/14/12 mature or diferentiated

Leukemia

ACUTE myelocytic leukemia ACUTE lymphocytic leukemia

CHRONIC myelocytic leukemia CHRONIC lymphocytic 6/14/12

Leukemia

ETIOLOGIC FACTORS

UNKNOWM Probably exposure to radiation Chemical agents Infectious agents Genetic Chemotherapy

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Leukemia
PATHOPHYSIOLOGY of ACUTE Leukemia Uncontrolled proliferation of immature cells suppresses bone marrow function severe anemia, thrombocytopenia and granulocytopenia

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Leukemia
PATHOPHYSIOLOGY of CHRONIC Leukemia Uncontrolled proliferation of DIFFERENTIATED cells slow suppression of bone marrow function milder symptoms

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Leukemia

ASSESSMENT FINDINGS ACUTE LEUKEMIA Pallor Fatigue Dyspnea Hemorrhages Organomegaly Headache vomiting
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Swollen lymph nodes that usually dont hurt Fevers or night sweats Infections Bleeding Weaknes Weight loss for no apparent reason Pain in the bones or joints Swelling or discomfort in the abdomen
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Leukemia

ASSESSMENT FINDINGS CHRONIC LEUKEMIA Less severe symptoms organomegaly

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Leukemia
LABORATORY FINDINGS Peripheral WBC count varies widely Bone marrow aspiration biopsy reveals a large percentage of immature cellsBLASTS Erythrocytes and platelets are decreased 6/14/12

Leukemia
Medical Management 1. Chemotherapy 2. Bone marrow transplantation

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Leukemia
Nursing Management 1. Manage AND prevent infection
Monitor temperature Assess for signs of infection Be alert if the neutrophil count drops below 1,000 6/14/12 cells/mm3

Leukemia
Nursing Management 2. Maintain skin integrity

3. Provide pain relief 4. Provide information as to therapy- chemo and bone marrow transplantation
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LYMPHOMA

Malignancies of the lymphoid tissues Characteized by proliferation of lymphpocytes, histocytes (macrophages) CAUSE: UNKNOWN

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LYMPHOMA

RISK FACTORS:

Genetic Immunosuppression (drug, transplantaion)

Viruses
Occupational herbicides/ chemical exposure

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LYMPHOMA

TYPES
Hodgkins lymphoma Non hodgkins lymphoma

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Hodgkins lymphoma

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Develops a single lymph node or chain of lymph nodes spreading to adjoining joints Involved lymph nodes that contains Reed-

Hodgkins lymphoma

MANIFESTATIONS

Lymphadenopathy Persistent fever Night sweats Fatigue weight loss Malaise Pruritus Anemia

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Non-Hodgkins lymphoma

Diverse group of lymphoid tissues malignancies that do not contain ReedSternberg cell More common Frequent in men that in

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Management

Chemotherapy Radiation Stem cell transplantion Staging

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MULTIPLE MYELOMA
Malignancy in which plasma cells multiply uncontrollably and infiltrate the bone marrow, lymph nodes spleen and other tissues PLASMA CELLS- Blymphoctes- produce antibodies CAUSE- unknown 6/14/12

manifestations

Bone pain Bence jones proteins in the urine Neurologic dysfunction

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Lethargy Consciousness Weakness

Management

Chemotherapy Stem cell transplantation Supportive care

Tx for hypercalcemia Hydration Biphosphate therapy*

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NURSING CARE

CHRONIC PAIN

Assess pain level and intensity Positioning Support with pillows Provide uninterrupted rest periods Teach non pharmacologic pain magmt Analgesic 6/14/12 Refer if pain is unrelieved

NURSING CARE

IMPAIRED MOBILITY

Change position q2 hours Provide a trapeze assist in repositioning

RISK FOR INJURY

Place needed items close at hand Provide safety measures to 6/14/12 prevent falls from bed.

DISORDERS OF THE THROMBOCYTES

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THROMBOCYTOPENIA

APC- <100,000 /ml Can lead to abnormal bleeding CAUSES

Decreased production Increased sequestrastion Accelerated destruction

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TYPES

PRIMARY- leads to increase platelt

Idiopathic thrombocytopenia purpura Thrombotic thrombocytopenia purpura

SECONDARY- may be caused by aplastuc anemia, bone marrow 6/14/12

ITP

Production of antibodies against the normal platelet Platelet destruction Decreased platelet count

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Manifestations

Petechiae and purpura

anterior trunk Arms Neck

Bruising Epistaxis Hematuria

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Thrombotic thrombocytopenia

Rare Thrombi occlude the arterioles and capillaries CAUSE: UNKNOWN

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MANIFESTATIONS

Purpura Petechiea Neurologic symptoms

Headache Seizures ALOCm

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Medications

Oral glucocorticoids Immuno suppressive drugs Heparin

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Management

Platelet transfusion Plasmaparesis Surgery

splenectomy

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Nursing care

Risk for bleeding Risk for thrombus formation

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DIC
Disruption of hemostasis characterized by widespread intravascular clotting and bleeding Devt of small blood clots inside the blood vessels throughout the body CAUSE; SEPSIS (most 6/14/12

Causes

Cancers Obstetric abruptio,pre ecclampsia,amniotic fluid embolism Massive tissue injurytrauma, burnss, extensive injury

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Manifestation
Frank hemorrhage from incision Oozing of blood from punctures, intravenous catheter sites Purpura, petechiae, bruising Cyanosis of extremities GI bleeding Dypnes, tachypnea, blood sputum Tachycardia, hypotension 6/14/12 Heamturia, oliguria, acute renal

Treatment

Careful monitoring of fluid balance Serial evaluation of coagulation parameters Sepsis- antibiotic Platelet transfusion

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HEMOPHILIA

Group of hereditary clotting factor d/o that lead to persistent and sometimes severe bleeding

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Types

Hemophilia A (classic hemophilia) Hemophlia B (Christmas disease) Von Willebrands disease Hemophilia C (Factor XI deficiency)

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Hemophilia A

Most common Deficiency or dysfunction of clotting factor VIII Transferred from MOTHER TO SON 1%-5% of normal>>severe

Spontaneouss bleeding Not related to injury Infrequent bleeding Due to trauma

1%-35%>>mild

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Hemophilia B

Christmas disease Deficiency in factor IX Hemophilia A and hemophilia B are clinically indentical

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Von willebrands disease

Most common hereditary bleeding disorder Defict or defective von willebrand factor- a protein that mediates platelet adhesion

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Hemophilia C

Factor XI deficiency Mild disorder Identified when post operative bleeding is prolonged

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Manifestations
Hemarthroses Easy bruising Bleeding from gums Prolonged bleeding following injuries GI bleeding (hematamesis, henatochesia) Hematuria 6/14/12 Intracranial hemorrhage.

Laboratory procedures

Coagulation tests Factor assays Amniocentesis

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Management

Fresh frozen plasma

Replaces all clotting factor except platelet Given to pt with hemophili A and von willlebrand disease prior to surgery

Desmopressin acetate

Factor IX concentrate Factor VIII concentrate 6/14/12

NURSING CARE

Monitor for any signs of bleeding Use safety measures Prevent from injury Avoid IM injections Dont give anticoagulants

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ciso

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