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Disorders of the Hematopoietic System
Chapter 37
Blood Disorders
� Blood Dyscrasias: abnormalities in the numbers and types of red blood
cells
� Coagulopathies: bleeding disorders
that involve platelets or clotting factors
Anemia
� Deficiency of erythrocytes or hemoglobin
� Reduction in amount of O2 carried to cells
� Causes� Blood loss
� Inadequate or abnormal erythrocyte production
� Destruction of normally formed RBCs
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AnemiaLab Interpretation
� MCV: size
� Microcytic
� Normocytic
� Macrocytic
� MCHC: color
� Hypochromic
� Normochromic
� Hyperchromic
� Hemoglobin
� Carries O2
� Hematocrit
� Volume of RBCs
Hypovolemic Anemia
� Occurs when there is a loss in blood volume
� Acute
� Chronic
� Assessment Findings
� Severe: EXTREME pallor, tachycardia, hypotension, ↓urine output, altered LOC
� Chronic: pallor, fatigue, chills, postural hypotension, tachycardia, tachypnea
� CBC: ↓Hgb, ↓Hct
Hypovolemic Anemia
� Medical Management
� IV fluids
� Blood transfusions
� Treatment of underlying conditions
� Nursing Management
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Hypovolemic Anemia
� Medical Management
� Blood transfusions
� Restore blood volume
� Nursing Management
� Vital signs
� Control acute bleeding
� Limit activity that increases O2
consumption
Iron Deficiency Anemia
� Insufficient iron for hemoglobin production
� Blood loss
� Inadequate dietary intake
� Malabsorption
� Hemolysis
Iron Deficiency Anemia
� Assessment Findings
� Reduced energy, fatigue
� Feel cold all the time
� Dyspnea with minor exertion
� Rapid heart rate
� Diagnostic Findings
� CBC, serum iron, blood smear
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Iron Deficiency Anemia
� Medical Management
� Iron: Oral, IM
� Iron rich foods in diet: meat, egg yolks, shellfish, whole grains, nuts, legumes
� Nursing Management
� Patient Teaching
�Dilute oral iron, drink through straw
�Orange juice to promote absorption
�Change in stool color
�Avoid antacid, coffee, tea, milk for 2 hr between iron use
Z-Track Technique
Sickle Cell Anemia
� Hereditary disease
� Primarily in African Americans; can occur in Meditarranean & Middle Eastern countries
� Sickle Cell Disease
� Inherit 2 defective genes (one from each parent); all Hgb is abnormal
� Sickle Cell Trait
� Inherit 1 defective gene
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Sickle Cell Anemia
Sickle Cell Crisis
� Vascular occlusions occur rapidly
under hypoxic conditions
� SX: Severe pain in ischemic tissue,
necrotic tissue, fever, swelling in joints
� Complications: Stroke, pulmonary
infarct, shock, renal failure
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Sickle Cell Anemia
Assessment Findings
� Jaundice
� Gallstones
� Predisposition to infection
� Chronic leg ulcers
� Priapism
� Acute chest syndrome
Diagnostic Findings
� Sickledex Test
� ↓ Hgb
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Sickle Cell Anemia
� Medical Management
� Supportive treatment
� Regular blood transfusions
� Bone marrow transplantation
� Antibiotic therapy
Sickle Cell Crisis
� Management
� Narcotic analgesia
� O2, bed rest
� IV fluids
� Desferal
Sickle Cell Anemia
Nursing Management
� Health history
� Physical assessment
� Patient teaching
� Adequate hydration
� Avoid vigorous exercise
� Avoid vasoconstriction, stop smoking
� Pneumonia vaccine
� Seek immediate medical attention for
signs of infection
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Hemolytic Anemia
� Erythrocytes are destroyed prematurily
� Spleen becomes obstructed and damaged resulting in an increased
risk of infection
� Persistent anemia causes:
� Tachycardia
� Dyspnea
� Cardiomegaly & dysrhythmias
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Hemolytic Anemia
� Assessment Findings
� Jaundice
� Enlarged spleen
� Shock
� Diagnostic Findings
� Microscopic examination
� Positive Coombs test
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Hemolytic Anemia
� Medical Management
� Remove the cause
� Corticosteroids
� Blood transfusion
� Splenectomy
� Nursing Management
� Health history
� Supportive care
Thalassemias
� Genetic disorder that results in abnormal Hgb synthesis
� Hereditary
� Alpha-thalassemias
� asymptomatic
� Beta-thalassemias
� Mild: asymptomatic
� Severe: Cooley’s anemia
Thalassemias
� Diagnosis
� Hgb electrophoresis
� Medical Management
� Frequent blood transfusions
� Cooley’s: iron chelating therapy
� Nursing Management
� Bedrest
� Protect from infection
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Pernicious Anemia
� Lack of intrinsic factor necessary for absorption of vitamin B12
� Assessment� Glossitis (inflammation of tongue)
� Stomatitis
� Digestive disturbances
� Neuromuscular changes
� Diagnostic findings� Schilling test
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Pernicious Anemia
� Medical Management
� Vitamin B12 injections for life
� Nursing Management
� If glossitis and stomatitis are present:
soft, bland diet
� Small frequent meals
� Oral care
Folic Acid Deficiency Anemia
� Characterized by immature erythrocytes
Four common causes
1. Poor nutrition
2. Malabsorption syndromes
3. Drugs that impede absorption & use of folic acid
4. Alcohol abuse & anorexia
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Folic Acid Deficiency Anemia
� Assessment Findings
� Similar to pernicious anemia
� Absence of neurologic problems
� Smooth, beefy-red tongue
� Medical Management
� Folic acid supplement
� Nursing Management
� Dietary choices high in folic acid
� Good oral hygiene
� Rest periods if fatigued easilyhttp://www.doctorspiller.com/images/OralAnatomy/AtrophicGlossitis.jpg
Polycythemia Vera
� Greater than normal number of erythrocytes, leukocytes & platelets
� Assessment Findings
� Reddish-purple face and lips
� Fatigue, dizziness, exertional dyspnea,
� Pruritis
� Excessive bleeding
� Hemorrhoids
� Splenomegaly
� Swollen joints
Polycythemia Vera
� Medical Management� Phlebotomy
� Anticoagulants
� Radiation therapy
� Antineoplastic drugs
� Nursing Management� Patient education
� Observe client for complications
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Leukemia
� Any malignant blood disorder in which rapid reproduction of leukocytes (usually immature) is unregulated
� Cause is unknown
� Predisposing factors
� Genetics
� Exposure to chemicals/radiation
� Viruses
Leukemia
� Hallmark Assessment Findings
� Infection
� Severe anemia with fatigue
� Easy bruising
� Diagnostic Findings
� ↓ leukocytes, erythrocytes, platelets
Leukemia
� Medical Management
� Antineoplastic drugs
� Transfusions
� Bone marrow & stem cell transplants
� Nursing Management
� Risk for infection
� Risk for hemorrhage
� Disturbed body image, anxiety, fear
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Multiple Myeloma
� Malignancy involving plasma cells (B-lymphocytes) in the bone marrow.
� Poor prognosis
� Survival of 1-5 years
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Multiple Myeloma
� Assessment Findings
� Skeletal pain
� Pathologic fractures
� Infection
� Anemia
� Diagnostic Findings
� X-ray: honeycomb bone lesions
� ↑ serum calcium, low blood counts
� Bence Jones protein
Multiple Myeloma
� Medical Management� Chemotherapy
� Radiation
� Bone marrow & stem cell transplant
� Analgesics
� Antibiotics
� Nursing Management� Pain
� Hydration
� Signs of infection
� Safety to prevent injury/fracture
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Agranulocytosis
� Most common cause: toxicity from drugs
� Antibiotics, anti-thyroid agents,
psychotropic agents, anti-convulsants
� Chemotherapy agents
� Assessment Findings� Infection, fever, chills
� General malaise, fatigue
� Opportunistic infections: mouth, throat, nose, rectum, and vagina
Agranulocytosis
� Medical Management� Removal of the cause
� Neupogen
� Nursing Management� Medication history
� Protective isolation if leukocyte count is low
� Good hand washing
� Restriction of visitors and staff
Aplastic Anemia
� Insufficient number of erythrocytes, leukocytes, and platelets
� Consequence of inadequate stem cell production in the bone marrow
� Exact cause is unknown
� Congenital: autoimmune
� Acquired: exposure to toxic chemicals,
radiation, and drug therapy
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Aplastic Anemia
� Assessment Findings
� Weakness, fatigue,
� Unusual bleeding, petechiae, ecchymosis
� Enlarged spleen
� Opportunistic infections
� Diagnostic Findings
� CBC: insufficient blood cells
� Bone marrow aspiration
Aplastic Anemia
� Medical Management� Withdrawal of the causative agent
� Transfusions
� Bone marrow & stem cell transplants
� Antibiotics
� Nursing Management� Assess for anemia, infection, bleeding
� Soft foods and oral hygiene to avoid bleeding from gums
Coagulopathies
� A term used to describe a condition in which a component that is
necessary to control bleeding is either missing or inadequate
� Examples:
� Thrombocytopenia
� Hemophilia
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Thrombocytopenia
� Platelet manufacture by bone marrow is decreased
� Aplastic anemia
� Hematologic
malignant
disorders
� Chronic alcoholism
� Chemotherapy or
radiation
� Platelet destruction by
spleen is increased
� Pregnancy
� Infection
� Drug induced
� Severe burns
� HIV
� Splenomegaly
Thrombocytopenia
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� Assessment Findings
� Purpura
� Bleeding
Thrombocytopenia
� Medical/Surgical Management
� Eliminate cause when possible
� Platelet transfusions
� Splenectomy
� Nursing Management
� Minimize risk for bleeding
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Hemophilia
� A disorder involving clotting factors
� Hereditary: sex-linked recessive
characteristic
� Three types� Hemophilia A (von Willebrand’s)
� Hemophilia B (Christmas disease)
� Hemophilia C (Rosenthal’s disease)
Hemophilia
Clinical manifestations:
� Slow, persistent bleeding from minor
trauma and small cuts
� Bleeding into joints
� Delayed bleeding after minor injury
� Uncontrollable hemorrhage after dental
extraction
� Epistaxis
� GI bleeding
� Hematuria
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