Ch. 9 Aplastic Anemia
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- Hypo-proliferative blood disorders (decreased cell production)
- Many different forms and causes
- Specific causes include Low Fe, B12, Folate an Erythropoietin.
Cause of Aplastic Anemia:
- 70% are idiopathic
- Iatrogenic (drug related): Insecticides, anti-cancer drugs
- Immune disorders
- Genetic disorders
Clinical features of aplastic anemia:
Survivors of aplastic anemias are at increased risk of other hematological diseases
- Bone marrow failure
- Acute or chronic
- Can be fatal or self-limiting
- Splenomegaly and lymphadenopathy absent
Decreased WBC, RBC, Platelets
Treatment of Aplastic Anemia:
- ID and remove toxic substance.
- Immuno-suppressant drugs
- Blood transfusions.
- Bone marrow transplants
: 70% fatality within 5 years
Normochromic-normocytic anemia (Aplastic 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.
- Congenital (present at birth)
Symptoms of Fanconi's Anemia:
- Microcephaly, mental retardation, short physical stature.
- Fatal by late teens.
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
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.
Long term treatment if treated with steroids
- Progressive anemia with normal WBC and Platelets
- Normochromic or slight macrocytosis.
- Increased Hemoglobin F production
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
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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