Chapter 49

Common Hematologic Disorders

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Disorders of Red Blood Cells

• Polycythemia
– Myeloproliferative disorder of increased red blood cell
(RBC) production
– Increase hematocrit and RBC mass
• Secondary polycythemia
– Disorder of increased RBC production, a normal
response to chronic hypoxia

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Assessment

• Assess for arterial and venous thrombosis.

• At increased risk for myocardial, cerebral infarction, deep
venous thrombosis, and pulmonary embolism
• History for cardiac or pulmonary disease
• Prior arterial or venous thrombosis
• Smoking history
• Frequent complaining of itching after a hot shower or
bath

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Management

• Serial phlebotomy
– 500 mL of blood is removed weekly until hematocrit
is <45%
• Monitor for development of iron deficiency.
• Hydroxyurea to suppress bone marrow
• Increased risk for acute leukemia
• Lower extremity compression devices
• Ambulate.
• Correct the underlying or secondary cause.
• Aspirin and dipyridamole do not reduce thrombotic
events.

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Anemias

• Blood loss anemia

• Hemolytic anemias
– Congenital hemolytic anemia
– Acquired hemolytic anemia
• Deficiency anemias
– Iron deficiency anemia
– Megaloblastic anemias
• Chronic anemia
• Aplastic anemia

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Assessment

• Assess for blood loss.

• Weakness, depressed mood, impaired cognitive
function, and easy fatigability
• Tachycardia, chest pain, dyspnea, and dizziness
• At risk for impaired tissue oxygenation, impaired organ
function, impaired susceptibility to thrombocytopenic
bleeding, increased risk of postoperative mortality,
increased probability of transfusion, and decreased
survival

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Physical Examination

• Pallor, tachycardia, hypotension, and signs of high-

output heart failure
• Splenomegaly, jaundice, and dark urine
• Intake and output
• Stool guaiac test
• Low serum iron

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Management

• Parenteral iron sulfate

• Blood transfusion
– When risks of decreased oxygen-carrying capacity
outweigh the risks of transfusion
– Complications: noninfectious, infectious, or
immunologic
• Erythropoietic-stimulating

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Nursing Care of the Patient With Anemia

• Assess for adverse effects of replacement therapy.

• Assess signs and symptoms indicative of decreased
perfusion.
• Identify the correct patient and ensure ABO
compatibility.
• Decrease metabolic needs and reduce
oxygen demand.
• Implement strategies to reduce phlebotomy
blood loss.

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Sickle Cell Disease

• Pathophysiology
– Chronic hereditary hemolytic anemia
– Blacks
– Abnormal hemoglobin, usually hemoglobin S (HbS)
– Elongated, sickle or crescent shaped, and rigid
– Causes inflammation, obstruction of the vessels, and
decreased delivery of oxygen

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Clinical Presentation

• Painful vaso-occlusive crisis

• Severe deep pain is present in the long bones of the
extremities.
• Severe pain resembling an acute abdomen
• Fever, malaise, and leukocytosis
• Microinfarctions of the heart, skeleton, spleen, and
central
nervous system (CNS)
• Acute chest syndrome is caused by pulmonary infarction
from fat embolism; bacterial infections.

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Management

• Aggressive intravenous fluid hydration

• Evaluate and treat infection.
• Oxygen
• Folic acid supplementation and hydroxyurea
• Around-the-clock narcotic dosing

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Disorders of White Blood Cells

• Leukopenia is an abnormally low number of WBCs

• Most common type is neutropenia.
• Clinical presentation
– Neutrophil count less than 1,500 cells/mm³, severe neutrophil count
of less than 200 cells/mm³ (agranuolcytosis)
– Susceptible to overwhelming infection and life-threatening sepsis
– Usual signs of the inflammatory response to infection may be
absent.
– Skin infections and ulcerative lesions of the mouth are
common.
– Respiratory tract is the most frequent site of serious
infection.

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Assessment

• History of viral infection or autoimmune diseases

• Medication history
• Presence of mouth ulcers, fever, shaking chills, and systemic
infection
• Splenomegaly
• Abnormal RBCs or WBCs
• Laboratory studies: hepatitis, HIV, and antinuclear antibody
(ANA)
• Bone marrow aspiration and biopsy

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Management

• Obtain appropriate cultures.

• Chest radiograph
• Broad-spectrum intravenous antibiotic therapy
• Investigate infection without fever.
• Antifungal agents if no response to antibiotics
• Hematopoietic growth factor (filgrastim, pegfilgrastim, or
sargramostim)
• Steroids
• IVIG

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Neoplastic Disorders

• Lymphoma
– Hodgkin lymphoma
– Non-Hodgkin lymphoma

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Clinical Presentation

• Symptoms include fever, fatigue, and weight loss

• Extensive chest disease, severe dyspnea
• Superior vena cava syndrome
• Obstruction of the bowel or the ureters
• Bone marrow involvement can lead to
– Decreased production of RBCs, WBCs, and platelets
– Impaired immune function and frequent, severe
infections
• Headaches, visual disturbances, motor dysfunction,
and
increased intracranial pressure
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Management

• The diagnosis, staging, and treatment done outpatient

• Manage complications.

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Leukemia

• Hematologic malignancies that affect bone marrow and

lymph tissues
• Acute lymphoblastic leukemia (ALL)
– Clones of immature lymphocytes proliferate and
replace normal cells.
• Acute myelogenous leukemia (AML)
– Abnormal production of the myeloid cell lines
(erythrocyte, neutrophil, megakaryocyte, and
macrophage)

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Clinical Presentation

• Headache
• Confusion
• CNS infarctions
• Acute respiratory insufficiency
• Pulmonary infiltrates

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Management

• Leukapheresis
• Hydroxyurea
• Chemotherapy

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Nursing Care of the Patient With a White
Blood Cell Disorder

• Monitor for infection.

• Oral care
• Monitor for diarrhea and consider Clostridium difficile
infection.

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Disorders of Hemostasis

• Platelet disorders
• Types of thrombocytopenia
– Drug-induced thrombocytopenia
– Heparin-induced thrombocytopenia
– Thrombotic thrombocytopenic purpura (TTP)
– Immune thrombocytopenic purpura (ITP)

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Assessment

• Assessment of symptoms associated with any of the risk

factors or associated disorders
• Patient or family history of bleeding
• Accurate medication and alcohol history
• Fatigue, fever, weight loss, or night sweats

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Physical Examination

• Examine the skin for petechiae and bruising.

• Examine oropharynx.
• Check stool for guaiac.
• Hypotension, tachycardia, and fever suggest sepsis.
• Enlarged spleen
• Signs of liver disease (jaundice, ascites, muscle
wasting)
• CBC with differential
• PT, PTT, fibrin degradation products
• Bone marrow biopsy
• HIV, HCV, and ANA screening

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Management

• Hold any drugs that may induce thrombocytopenia.

• Avoid aspirin, antiplatelet agents, and NSAIDs.
• Avoid trauma.
• Daily labs
• Platelet transfusions
• Plasmapheresis
• Antiplatelet agents and prednisone
• Corticosteroids are the initial therapy for ITP.
• IVIG
• Pneumococcal, meningococcal, and Haemophilus influenzae
type B vaccines

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Coagulation Disorders

• von Willebrand’s disease

• Hemophilia A
• Management
– Correct the coagulation factor deficiency and treat
sequelae.
– Intravenous or nasal administration of desmopressin
acetate
• Hemophilia A or active bleeding requires intravenous
infusion
of factor VIII concentrate.
• von Willebrand’s disease requires factor VIII concentrate
that
contains von Willebrand’s factor.

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Disseminated Intravascular Coagulation

• Imbalance between the natural procoagulant and anticoagulant

systems
• Unregulated thrombin activity, microvasculature thrombi,
platelet consumption, and microangiopathic hemolytic anemia
• Etiology
– DIC is a secondary complication.
– Extrinsic pathway is activated by damage to the
endothelial lining of blood vessels.
– Intrinsic pathway is activated when subendothelial tissue is
exposed to the bloodstream and circulating factor XII
comes in contact with the exposed tissue.

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Clinical Presentation

• Systemic ischemia from the thrombi formation

• Minor or major hemorrhage

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Assessment

• Complete history
• Acute or chronic presentation
• Low-grade bleeding
• Unexpected thrombotic events
• Signs and symptoms of inappropriate clotting
• Demarcation cyanosis
• Bleeding from the nose, gums, lungs, gastrointestinal
tract, surgical sites, injection sites, and intravascular
access sites; hematuria; petechial rashes; and purpura
fulminans

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Care of the Patient

• Onset is sudden and acute.

• Constant reassessment
• Dyspnea
• Hypotension
• Ischemic bowel
• Signs and symptoms of the onset of shock

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Laboratory Studies

• Platelets
• Fibrinogen
• PT
• PTT

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Management

• Eliminate the causative agent.

• Antibiotic or antifungal therapy for sepsis
• Antineoplastic therapy
• Fluid replacement
• Oxygen
• Resolution of low-flow states
• Heparin therapy
• FFP, cryoprecipitate, RBC

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