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13 Cards in this Set
- Front
- Back
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Definition of Aplastic Anemia
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-immunologic or idiopathic suppression of blood cell progenitor prolif & maturation --> anemia, thrombocytopenia, and leukemia
-*can be common presentation of several etiologies |
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****Hallmarks of Aplastic Anemia
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REQUIREs BOTH:
1. pancytopenia (not enough of all cells) 2. Acellular BM filled w/ fat -sustained -caused by stem cell injury |
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Stem cell injury can result from?
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1. accum effects of mult noxious exposures to pluripot stem cells
2. Defect in stem cell |
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Defects of Stem Cell
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-proliferation of single abnormal cell (clone) which hinders production of other stem cells (immune)
-impaired prod or fxn of cytokines (growth factors) -fail of stomal microenvironment |
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Single Cell (Monoclonal) AA
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-Genetic predisposition assoc w/ HLA type (DR-2)
-overalp w/ other monoclonal anemias: PNH, Hypoplastic myelodysplastic syndrome, all lead to AML as end stage disease |
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What are symptoms of ALL anemias?
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1. fatigue
2. SOB w/ exertion 3. Rapid HR 4. Pale skin |
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What are symptoms of aplastic anemia?
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-freq/prolonged infx
-unexplained/easy bruising -nosebleeds and gums -skin rash -dizziness -headache |
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Lab evaluation of Aplastic Anemia
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1. pancytopenia
2. Acellular (or marked hypocellular) BM 3. Decreased CFU-GM and BFU-E 4. Dec long term culture initiating cells to less than 1% 5. R/O ALL or Hairy cell anemia |
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***Acellular (or marked hypocellular) BM Lab evaluation of Aplastic Anemia
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-marrow shows numerous spicules w/ empty fatty space
-few hematopoetic cells -lymphos, plasmas, and macros -no other causes of pancytopenia are noted |
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Differential Diagnosis of Aplastic Anemia
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-other conditions that may infiltrate, replace, supress BM production
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Tx of Aplastic Anemia
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1. Remove offending agent
2. Blood transfusions supoort 3. BM transplantation 4. Immunosuppressants (cyclosporine, anti-thymocyte globin) 5. Corticosteroids 6. BM stimulants |
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Prognosis of Aplastic Anemia
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-prev to BM trans & immunosuppress most died w/in 1 yr
-Now - 75% cure -But 25% severe graph vs host dis -15% relapse -25% progress to PNH, MDS, AML |
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***What diagnostic test would provide the greatest likelihood of making a definitive diagnosis?
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-BM aspirate and biopsy
**shows numerous spicules w/ empty fatty space, few hematopoeitic cells, lymphos, plasmas, macros present |
