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Assessment and Management of Patients With Hematologic Disorders

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Question
Is the following statement True or False? Hematopoiesis is the complex process of the formation and maturation of blood cells.

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Answer
True Hematopoiesis is the complex process of the formation and maturation of blood cells.

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Hematologic System
The blood and the blood forming sites, including the bone marrow and the reticuloendothelial system Blood

Plasma
Blood cells Hematopoiesis

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Blood Cells
ErythrocyteRBC LeukocyteWBC Neutrophil Monocyte Eosinophil Basophil LymphocyteT lymphocyte and B lymphocyte Thrombocyteplatelet
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Hematopoiesis

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Hemostasis
Refer to fig. 33-3

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Question
What type of anemia results from red blood cell destruction? A.Bleeding

B.Hemolytic
C.Hypoproliferative D.None of the above

Copyright 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins

Answer
B Bleeding results from red blood loss. Hemolytic anemia results from red blood cell destruction. Hypoproliferative anemia results from defective red blood cell production.

Copyright 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins

Anemias
Lower than normal hemoglobin and fewer than normal circulating erythrocytes. A sign of an underlying disorder Hypoproliferative: defect in production of RBCs Due to iron, vitamin B12, or folate deficiency, decreased erythropoietin production, cancer Hemolytic: excess destruction of RBCs

Due to altered erythropoiesis, or other causes such as hypersplenism, drug-induced or autoimmune processes, mechanical heart valves
May also be due to blood loss
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Manifestations
Depend upon the rapidity of the development of the anemia, duration of the anemia, metabolic requirements of the patient, concurrent problems Fatigue, weakness, malaise Pallor or jaundice Cardiac and respiratory symptoms

Tongue changes
Nail changes Angular cheilosis

Pica
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Medical Management
Correct or control the cause Transfusion of packed RBCs Treatment specific to the type of anemia Dietary therapy Iron or vitamin supplementationiron, folate, B12 Transfusions Immunosuppressive therapy Other
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Nursing Process: The Care of the Patient with AnemiaAssessment


Health history and physical exam

Laboratory data
Presence of symptoms and impact of those symptoms on patients life; fatigue, weakness, malaise, pain Nutritional assessment Medications Cardiac and GI assessment

Blood lossmenses, potential GI loss


Neurologic assessment
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Nursing Process: The Care of the Patient with AnemiaDiagnoses


Fatigue Altered nutrition Altered tissue perfusion Noncompliance with prescribed therapy

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Collaborative Problems/Potential Complications


Heart failure Angina Paresthesias Confusion

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Nursing Process: The Care of the Patient with AnemiaPlanning


Major goals include 1. decreased fatigue,

2. attaining or maintaining adequate nutrition,


3. maintaining adequate tissue perfusion, 4. complying with prescribed therapy, and 5. the absence of complications.
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Interventions Balance physical activity, exercise, and rest. Maintain adequate nutrition.

Patient education to promote compliance with medications and nutrition.


Monitor VS and pulse oximetry, provide supplemental oxygen as needed. Monitor for potential complications.
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Question
Is the following statement True or False? Leukocytosis refers to a decreased level of leukocytes in the circulation.

Copyright 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins

Answer
False Leukocytosis refers to an increased level of leukocytes in the circulation. Leukopenia refers to a decreased level of leukocytes in the circulation.

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Leukemia
Hematopoietic malignancy with unregulated proliferation of leukocytes Types:

Acute myeloid leukemia


Chronic myeloid leukemia Acute lymphocytic leukemia

Chronic lymphocytic leukemia

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Acute Myeloid Leukemia (AML)


Defect in stem cell that differentiate into all myeloid cells: monocytes, granulocytes, erythrocytes, and platelets
Most common nonlymphocytic leukemia Affects all ages with peak incidence at age 67 Prognosis is variable and wide range Manifestations: fever and infection , weakness and fatigue, bleeding tendencies, pain from enlarged liver or spleen, hyperplasia of gums, bone pain Treatment aggressive chemotherapyinduction therapy, BMT or PBSCT

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Chronic Myeloid Leukemia (CML)


Mutation in myeloid stem cell with uncontrolled proliferation of cells
Chromosome 22 (Philadelphia chromosome) has a section of DNA missing

Stages:
1. chronic phase, life expectancy >5 yrs 2. transformational phase

3. blast crisis (acute phase), survival less than 6 mths


Uncommon in people under 20, with increased incidence with age. Mean age 67

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Chronic Myeloid Leukemia (CML)


Manifestations Initially may be asymptomatic 1

Confusion or shortness of breath 2


Enlarged, tender spleen, or enlarged liver Malaise, anorexia, weight loss,

Treatment
Imatinib mestylate (Gleevec) 3 Chemotherapy, BMT or PBSCT
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Acute Lymphocytic Leukemia (ALL)


Uncontrolled proliferation of immature cells (lymphoblasts) from lymphoid stem cell Most common in young children, boys more often than girls, 4 yrs old is peak Prognosis is good for children, but diminishes with age

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Acute Lymphocytic Leukemia (ALL)


Manifestations

Immature lymphocytes proliferate in the marrow1


Large liver or spleen and bone pain Headache, vomiting 2

Treatment - complex
Chemotherapy Corticosteroids

imatinib mestylate (if Philadelphia chromosome positive)


BMT or PBSCT
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Chronic Lymphocytic Leukemia (CLL)


Common among older adults , ave. age 72 Results form malignant B lymphocytes 1

May escape apoptosis 2, resulting in excessive accumulation of cells


Survival varies from 3-15 years Lymphadenopathy Hepatomegaly Splenomegaly In later stages anemia and thrombocytopenia Autoimmune complications occur 1
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Chronic Lymphocytic Leukemia (CLL)


Manifestations

Some pts asymptomaic 1


Lymphadenopathy 2 Splenomegaly

Some develop B symptoms 3


Infections are common Treatment

early stage may require no treatment 4


Chemotherapy given with monoclonal antibody therapy 5
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Nursing Process: The Care of the Patient with LeukemiaAssessment


Health history Assess symptoms of leukemia, and for complications of anemia, infection, and bleeding

Weakness and fatigue


Laboratory tests CXR, Leukocyte count, ANC, hematocrit, platelets (may be very low)1 electrolytes, culture reports

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Nursing Process: The Care of the Patient with LeukemiaDiagnoses


Risk for bleeding and infection Risk for impaired skin integrity

Impaired gas exchange


Impaired mucous membrane Imbalanced nutrition Acute pain Hyperthermia Fatigue and activity intolerance Impaired physical mobility
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Nursing Process: The Care of the Patient with LeukemiaDiagnoses


Risk for excess fluid volume Diarrhea Risk for deficient fluid volume

Self-care deficit
Anxiety Disturbed body image Potential for spiritual distress Grieving diagnoses Deficient knowledge
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Collaborative Problems/Potential Complications


Infection Bleeding Renal dysfunction

Tumor lysis syndrome


Nutritional depletion Mucositis

Depression

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Nursing Process: The Care of the Patient with Leukemia- Planning


Major goals absence of complications Attaining and maintaining adequate nutrition Activity tolerance Ability for self-care Ability to cope with diagnosis and prognosis Positive body image Understanding disease process and its treatment.
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Interventions
Interventions related to risk of infection and bleeding Mucositis Frequent, gentle oral hygiene Soft toothbrush, or if counts are low, sponge-tipped applicators Rinse only with NS, NS and baking soda, or prescribed solutions Perineal and rectal care

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Improving Nutrition
Oral care before and after meals Administer analgesics before meals Appropriate treatment of nausea Small, frequent feedings Soft foods that are moderate in temperature Low-microbial diet Nutritional supplements

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Lymphoma
Neoplasms of lymph origin Hodgkins lymphoma Non-Hodgkins lymphoma

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Hodgkin Lymphoma
Relatively rare More men than women, Two peaks 20s and 50s, familial pattern

Unicentric origin, malignant cell is ReedSternberg cell1


Excellent cure rate with treatment Cause is unknown thought to be viral

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Hodgkin Lymphoma
Manifestations

Painless, firm lymph node enlargement on one side of neck


Pruritis B symptoms Sed rate (ESR erythrocyte sedimentation rate) Treatment Determined by stage of the disease May include chemotherapy and/or radiation therapy
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Hodgkins Lymphoma Nursing Management


Encourage patient to reduce other factors that increase the risk of developing second cancers

1. Tobacco use
2. Alcohol use 3. Exposure to carcinogens

4. Exposure to sunlight
Screening for effects of treatment Provide education regarding self care strategies Provide education regarding disease management
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Non-Hodgkin Lymphoma (NHL)


Lymphoid tissues infiltrated with malignant cells Spread is unpredictable Incidence increases with age, with average age 67 Incidence increases with autoimmune disorders Prognosis varies

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Non-Hodgkin Lymphoma (NHL)


Manifestations Symptoms are variable Lymphadenopathy most common 1 Symptoms may be absent or minor until diagnosed at later stage (III or IV) when symptomatic B symptoms

Treatment
Determined by type and stage of disease 2 May include interferon, chemotherapy, and/or radiation therapy.
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Non-Hodgkin's Lymphoma (NHL)


Diagnostic Findings Staging based on data from CT and PET scans, bone marrow biopsies, sometimes cerebrospinal fluid

Stage based on site of disease and its spread to other sites

Copyright 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins

Question
What should any elderly patient be evaluated for whose chief complaint is back pain and has an elevated total protein level? A. Anemia B. Leukemia C. Multiple myeloma D. Non-Hodgkins lymphoma

Copyright 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins

Answer
C Any elderly patient whose chief complaint is back pain and has an elevated total protein level should be evaluated for possible myeloma.

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Multiple Myeloma
Malignant disease of plasma cells in the bone marrow with destruction of bone
Prognosis based on markers 1. Serum albumin

2. Serum beta2 microglobulin

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Multiple Myeloma
Manifestations Bone pain (back or ribs) Osteoporosis & spinal fractures

Hypercalcemia 1
Renal failure Anemia 2, so fatigue , weakness

Reduced leukocytes and platelets 3


Treatment Chemotherapy, corticosteroids, radiation therapy, BMT or PBSCT, biphosphonates (strenghten the bone)
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Bleeding Disorders
Disseminated Intravascular Coagulation (DIC) Primary Thrombocythemia Thrombocytopenia Idiopathic Thrombocytopenia Purpura (ITP)

Hemophilia
Acquired Coagulation disorders: liver disease, anticoagulants, vitamin K deficiency Bleeding precautions

Copyright 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins

Question
Is the following statement True or False? Disseminated intravascular coagulation is not a disease but a sign of an underlying condition.

Copyright 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins

Answer
True Disseminated intravascular coagulation is not a disease but a sign of an underlying condition.

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DIC
Severity is variable; may be life-threatening -80% mortality rate

Triggers may include sepsis, trauma, shock, cancer, abruptio placenta, toxins, and allergic reactions.
Hemostasic mechanism is altered causing massive clotting. As clotting factors are consumed, bleeding occurs. Symptoms are related to tissue ischemia and bleeding. 1 Laboratory tests. Treatment: treat underlying cause, correct tissue ischemia, replace fluids and electrolytes, maintain blood pressure, replace coagulation factors, use heparin.

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DIC
Manifestations Organ failure (or compromised function) Low platelet and fibrinogen levels 1

Prolonged PT, PTT, INR


Elevated D Dimer Bleeding from minimal to hemorrhage from all orifices and sites 2 Management Treat underlying cause

Correct secondary effects

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Nursing Process: The Care of the Patient with DICAssessment


Be aware of patient who are at risk for DIC and assess for signs and symptoms of the condition. Assess for signs and symptoms and progression of thrombi and bleeding.

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Nursing Process: The Care of the Patient with DICDiagnoses


Risk for fluid volume deficient Risk for impaired skin integrity Risk for imbalanced fluid volume Ineffective tissue perfusion Death anxiety

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Collaborative Problems/Potential Complications


Renal failure Gangrene Pulmonary embolism or hemorrhage Acute respiratory distress syndrome Stroke

Copyright 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins

Nursing Process: The Care of the Patient with DICPlanning


Major goals maintenance of hemodynamic status, maintenance of intact skin and oral mucosa, maintenance of fluid balance, maintenance of tissue perfusion, enhanced coping, and absence of complications.

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Interventions
Assessment and interventions should target potential sites of organ damage. Monitor and assess carefully

Avoid trauma and procedures which increase risk of bleeding, including activities which would increase intracranial pressure.

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Therapies for Blood Disorders


Anticoagulant therapy Splenectomy Therapeutic apheresis Therapeutic phlebotomy Blood component therapy *** see handout

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Complications of Transfusions
Febrile nonhemolytic reaction Acute hemolytic reaction Allergic reaction Circulatory overload Bacterial contamination Transfusion related acute lung injury Delayed hemolytic reaction Disease acquisition Complications of long-term transfusion therapy
Copyright 2010 Wolters Kluwer Health | Lippincott Williams & Wilkins

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