Ch. 9 Aplastic Anemia

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Angela6
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199475
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Ch. 9 Aplastic Anemia
Updated:
2013-02-10 17:58:09
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Hematology
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Aplastic Anemia
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  1. Aplastic Anemia:
    • Hypo-proliferative blood disorders (decreased cell production)
    • Many different forms and causes
    • Specific causes include Low Fe, B12, Folate an Erythropoietin.
  2. Cause of Aplastic Anemia:
    • 70% are idiopathic
    • Iatrogenic (drug related): Insecticides, anti-cancer drugs
    • Radiation
    • Immune disorders
    • Infections
    • Genetic disorders
  3. Clinical features of aplastic anemia:
    • Bone marrow failure
    • Pancytopenia
    • Acute or chronic
    • Can be fatal or self-limiting
    • Splenomegaly and lymphadenopathy absent

    Survivors of aplastic anemias are at increased risk of other hematological diseases
  4. Define pancytopenia:
    Decreased WBC, RBC, Platelets
  5. Treatment of Aplastic Anemia:
    • ID and remove toxic substance.
    • Immuno-suppressant drugs
    • Blood transfusions.
    • Bone marrow transplants

    Poor prognosis: 70% fatality within 5 years
  6. Normochromic-normocytic anemia (Aplastic Anemia)
  7. Fanconi's Anemia:
    • Recessive genetic disorder (disease gene inherited from both parents)
    • Found in all ethnic groups
    • Prevents cells from fixing damaged DNA or removing toxic, oxygen free radicals that damage cells.
    • Pancytopenia
    • Congenital (present at birth)
  8. Symptoms of Fanconi's Anemia:
    • Microcephaly, mental retardation, short physical stature.
    • Fatal by late teens.
  9. Pure Red Cell Aplasia (PRCA):
    • Autoimmune attack on RBC HPCs
    • 50% of cases associated with Thymomas

    • Decreased RBC counts (normal WBC and Platelet counts)
    • Increased erythropoietin production.

    Most common in middle age
  10. Blackfan- diamond syndrome:
    • Autosomal dominant genetic/congenital RBC aplasia.
    • Disease condition if gene inherited from only one parent.
    • 1:200,000 births
    • Diagnosed within 1st year.

    • Progressive anemia with normal WBC and Platelets
    • Normochromic or slight macrocytosis.
    • Increased Hemoglobin F production

    Long term treatment if treated with steroids
  11. Transient Erythroblastopenia of Children (TEC):
    • Acute anemia in previously healthy children (< 8 years old) following a viral infection.
    • Immune mediated attack on RBC HPCs

    • Median diagnosis age: 2 year old
    • Self-limiting recovery in 1-2 months

    • Normocytic-normochromic RBCs
    • Initial decrease, then increased Retic count
  12. Congenital Dyserythropoietic Anemia (CDA):
    • Diverse group of genetic disorders (Types I, II, III)
    • Type II most common: Autosomal recessive

    Ineffective erythropoiesis with unusual mult-nucleated RBC HPC

    A combo of drug/and viral damage and subsequent immune attack.

    • Increased RBC destruction in the marrow
    • Increased plasma bilirubin

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