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Assessment and
Management of
Patients With
Hematologic Disorders
Copyright © 2008 Lippincott Williams & Wilkins.
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Hematologic System
• The blood and the blood forming sites, including the
bone marrow and the reticuloendothelial system
• Blood
– Plasma (fluid portion of blood -55%)
– Blood cells
• Hematopoiesis
– The body replenishes its supply of blood cells
– Primary site is bone marrow
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Blood Cells
• Erythrocyte: RBC
• Leukocyte: WBC
– Neutrophil
– Monocyte
– Eosinophil
– Basophil
– Lymphocyte: T lymphocyte and B lymphocyte
• Thrombocyte: platelet
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Hematologic Studies
• CBC
• Peripheral smear
• Bone marrow aspiration and biopsy
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Blood Smear
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Hemostasis
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Polycythemia
• Increased volume of erythrocytes (HCT >55%M/>50%F)
• Primary
– Proliferative disorder - bone marrow is hypercellular
– Patients at increased risk for thromboses and bleeding (due to
platelet dysfunction)
– Managed usually via phlebotomy
• Secondary
– Caused by excessive prodcution of erythropoietin
• May be d/t hypoxic stimulus, neoplasms (epo produced by malignancy)
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Polycythemia
• Manifestations
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Hypervolemia and hyperviscosity
Ruddy complexion
Hypertension
Headache
Dizziness, tinnitus
Signs of embolization
• Management
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Phlebotomy - reduce HCT to 45% or less
Hydration
Avoid iron supplementation
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Neutropenia
• Absolute neutrophil count
– WBC x %neutrophils
– <1500/ ul (severe if <500/ul)
• Predisposed to infection
– Neutropenia may mask clasic signs of infection - e.g.,
even low grade fever may be of great significance
– May progress rapidly to sepsis
• Nursing and collaborative care
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Determine cause
Identify offending organism
Institute prophylactic or therapeutic antibiotic
Administer hematopoetic growth factors
Institute protective environmental measures
See table 31-22 and 31-23
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Leukemia
• Hematopoietic malignancy with unregulated
proliferation of leukocytes
• Affect blood and blood-forming tissues of bone
marrow, lymph system, and spleen
• Types (see table 31-24)
– Acute myeloid leukemia
– Chronic myeloid leukemia
– Acute lymphocytic leukemia
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– Chronic lymphocytic leukemia
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Acute Myeloid Leukemia (AML)
• Defect in the stem cells that differentiate into all
myeloid cells: monocytes, granulocytes,
erythrocytes, and platelets
• Most common leukemia in adults
• Affects all ages with peak incidence at age 60
• Abrupt onset
• Manifestations: fever and infection, weakness
and fatigue, bleeding tendencies, pain from
enlarged liver or spleen, hyperplasia of gums,
and bone pain
• Treatment is aggressive chemotherapy:
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induction therapy, BMT, and PBSCT
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Chronic Myeloid Leukemia (CML)
• Mutation in myeloid stem cell with uncontrolled
proliferation of cells: excessive neoplastic granulocyts
• Stages: chronic phase, blast crisis
• Uncommon in people under 20; incidence increases
with age; mean age is 55 to 60 years
• Life expectancy is 3 to 5 years
• Manifestations (initially may be asymptomatic):
malaise; anorexia; weight loss; sternal pain; enlarged,
tender spleen; enlarged liver
• Treatment: chemotherapy, BMT, and PBSCT, radiation
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Acute Lymphocytic Leukemia
• Uncontrolled proliferation of immature cells from
lymphoid stem cell
• Most common in young children
• Prognosis is good for children; 80% event-free after
5 years, but survival drops with increased age
• Manifestations: fever, bleeding, anemia, mouth
sores, symptoms of meningeal involvement and
liver, spleen, and bone marrow pain
• Treatment: chemotherapy, BMT or PBSCT
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Chronic Lymphocytic Leukemia
• Malignant B lymphocytes, most of which are
mature, may escape apoptosis, resulting in
excessive accumulation of cells
• Most common form of chronic leukemia in
adults
• More common in older adults and affects
men more often
• Survival varies from 2 to 14 years depending
upon stage
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Chronic Lymphocytic Leukemia
(cont.)
• Manifestations: lymphadenopathy,
fatigue, anorexia, hepatomegaly,
splenomegaly; in later stages, anemias
and thrombocytopenia;; “B symptoms”
include fever, night sweats, and weight
loss
• Treatment: early stage may require no
treatment, chemotherapy,radiation
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Chemotherapy
• Induction therapy
– Nursing implications
• Intensification therapy
• Consolidation therapy
• Maintenance therapy
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Nursing Process—Assessment of
the Patient With Leukemia
• Health history
• Assess for symptoms of leukemia and
complications of anemia, infection, and
bleeding
– Weakness and fatigue
– See table 31-27
• Laboratory tests
– Leukocyte count, ANC, hematocrit, platelets,
electrolytes, and cultures reports
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Nursing Process—Diagnosis of
the
Patient With Leukemia
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Risk for bleeding
Risk for impaired skin integrity
Impaired gas exchange
Impaired mucous membrane
Imbalanced nutrition
Acute pain
Fatigue and activity intolerance
Risk for infection
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Nursing Process—Diagnosis of
the
Patient With Leukemia (cont.)
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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
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Infection
Bleeding
Renal dysfunction
Tumor lysis
syndrome
• Nutritional
depletion
• Mucositis
• Depression
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Nursing Process—Planning the
Care of the
Patient With Leukemia
• Major goals include absence of complications,
attainment and maintenance of adequate
nutrition, activity tolerance, ability for self-care
and to cope with the diagnosis and prognosis,
positive body image, and an understanding of
the disease process and its treatment
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Interventions
• Reduce risk of infection
• Reduce risk of bleeding
• Mucositis
– Frequent, gentle oral hygiene
– Soft toothbrush, or if counts are low, spongetipped applicators
– Rinse only with NS, NS and baking soda, or
prescribed solutions
– Perineal and rectal care
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Improving Nutrition
• Provide oral care before and after meals
• Administer analgesics before meals
• Provide appropriate treatment of nausea
• Provide small, frequent feedings with soft
foods that are moderate in temperature
• Provide a low-microbial diet
• Provide nutritional supplements
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Lymphoma
• Neoplasm originating in the bone
marrow and lymphatic structures
resulting in the proliferation of
lymphocytes
• Hodgkin’s lymphoma
• Non-Hodgkin’s lymphoma
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Hodgkin’s Disease
• Unicentric origin
• Reed–Sternberg cell
• Suspected viral etiology (EBV); familial pattern;
incidence occurs in early 20s and again after age
50
• Excellent cure rate with treatment
• Manifestations: painless lymph node
enlargement; pruritus; B symptoms : fever, night
sweats, and weight loss
• Treatment is determined by stage of the disease
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and may include chemotherapy and/or radiation
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Non-Hodgkin's Lymphoma (NHL)
• Lymphoid tissues become infiltrated with
malignant cells that spread unpredictably;
localized disease is rare
• Incidence increases with age; the average age
of onset is 50 to 60
• Prognosis varies with the type of NHL
• Diagnosed via lymph node biopsy and imaging
• Treatment is determined by type and stage of
disease and may include interferon,
chemotherapy, and/or radiation therapy
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Multiple Myeloma
• Malignant disease of plasma cells in the bone
marrow with destruction of bone
• M protein and Bence-Jones protein
• Median survival is 3 to 5 years; there is no cure
• Manifestations: insidious onset; bone pain,
osteoporosis, fractures, elevated serum protein
hypercalcemia, renal failure, symptoms of
anemia, fatigue, weakness, increased serum
viscosity, and increased risk for bleeding and
infection
• Treatment may include chemotherapy,
corticosteroids, stem cell transplant, and
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biphosphonates
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Multiple Myeloma
• Nursing care is relates to bone involvement
and sequelae from bone breakdown
– Hypercalcemia
– Fractures
– Pain
– Infection
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Bleeding Disorders
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Thrombocytopenia
Idiopathic thrombocytopenia purpura (ITP)
Hemophilia
Acquired coagulation disorders: liver
disease, anticoagulants, and vitamin K
deficiency
• Disseminated intravascular coagulation
(DIC)
• Bleeding precautions
– See Nursing care plan 31-2, table 31-15
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Hemophilia
• Inherited bleeding disorder, results in
deficiency of factor VIII or IX; varying degrees
of disease
• Manifested by hemorrhage into various body
parts
• Medical management includes FFP
transfusion, recombinant clotting factors,
desmopressin (DDAVP)
• Nursing management: control bleeding, patient
education aimed at prevention of bleeding
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Disseminated Intravascular
Coagulation
• Not a disease but a sign of an underlying disorder;
bleeding/thrombotic disorder
• Severity is variable; may be life-threatening
• Triggers may include sepsis, trauma, shock,
cancer, abruptio placentae, toxins, and allergic
reactions, malignancies, liver disease
• Altered hemostasis mechanism causes massive
clotting in microcirculation; as clotting factors are
consumed and lost, bleeding occurs; symptoms
are related to tissue ischemia and bleeding
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Disseminated Intravascular
Coagulation
• Manifestations include simultaneous bleeding and
thrombosis;
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Pallor, petechiae, purpura, oozing, bleeding, hematomas,
cyanosis, tissue necrosis…
• Lab tests - prolonged PT/PTT, elevated d dimer
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See table 31-20
• Treatment: treat underlying cause, correct tissue ischemia,
replace fluids and electrolytes, maintain blood pressure,
replace coagulation factors, and use heparin
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Heparin Induced
Thrombocytopenia
• Complication of heparin therapy
– Higher risk with prolonged use
• Hallmark sign is decline in platelet count,
usually after 4-14 days (usually drops by 50%
or more)
• Patients at risk for bleeding; also for
thrombosis (venous or arterial)
• Treatment includes cessation of heparin,
institution of alternative anticoagulation;
follow platelet count
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• Review Heparin, Low Molecular Weight
Heparin, and Warfarin therapies
– Pharmacology book
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Nursing Process—Assessment of
the Patient With DIC
• Be aware of patients 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—Diagnosis of
the
Patient With DIC
• Risk for fluid volume deficiency
• 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
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Nursing Process--Planning the
Care of the Patient With DIC
• Major goals include 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 /actual sites of organ damage
• Monitor and assess carefully for bleeding and
thrombosis
• Avoid trauma and procedures that increase
the risk of bleeding, including activities that
increase intracranial pressure
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Blood Transfusion Administration
• Review patient history including history of
transfusions and transfusion reactions; note
concurrent health problems and obtain baseline
assessment and VS
• Perform patient teaching and obtain consent
• Equipment: IV (19-20 gauge or greater for PRBCs),
appropriate tubing, and normal saline solution
• Procedure to identify patient and blood product
• Monitoring of patient and VS
• Postprocedure care
• Nursing management of adverse reactions
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Complications
see table 31-33!
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Febrile nonhemolytic reaction
Acute hemolytic reaction
Allergic reaction (mild or severe)
Circulatory overload
Bacterial contamination
Transfusion-related acute lung injury
Delayed hemolytic reaction
Disease acquisition
Complications of long-term transfusion
therapy
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Alternative to blood
transfusion
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Growth factors
Erythropoietin
Granulocyte-Colony Stimulating Factor
Granulocyte-Macrophage Colony
Stimulating Factor
• Thrombopoeitin
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Peripheral Blood Stem Cell
Tranplantation and Bone Marrow
Tranplantation
• Autologous stem cell transplantation uncommon
• Donor tranplantation - marrow stem cells or
bone marrow from a donor are transfused
– First, patient undergoes intense chemo or radiation
to ablate the patient’s own marrow
– Success depends on tissue compatibility and the
patient’s tolerance of the immunosuppression
• Nursing management is focused on prevention
of infection and early detection of complication
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