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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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Hemophilia

� Medical Management

� Transfusions

� Thrombin application

� Direct pressure/cold compress/ice to bleeding site

� Nursing Management

� Health history, last bleeding episode

� Patient teaching