Term
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Definition
-hypoproliferative disorder, characterized by reduced growth or production of blood cell (unusual hematologic disease, either acquired/congenital in etiology)
-ex. Fanconi |
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Term
| Compare & contrast aplastic anemia w/ other types of pancytopenias |
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Definition
aplastic anemia- all cell lines decreased, RBC typ. normocytic normochromic, ex. Fanconi- inherited, seen w/ in 1st 10 yrs. of life, predisposition to have bone marrow failure
RBC hypoproliferation (only involve RBC line):
Diamond-Blackfan syndrome- inherited, rare, defect in stem cell->unable to make RBC, usu. diagnosed b/w 1-6 yrs.
-all decrease except pronormoblast
Acquired- usu. ass. w/ cancer of thymus-> blocking production of RBCs
Acute transient- usu. seen in healthy children/viral infect (w/ in 3m)/humoral inhibition/transient-->goes away |
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Term
| List the criteria of diagnosis of aplastic anemia |
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Definition
| -made when at least 2 of the 3 peripheral blood values fall below critical levels (erythrocytes, leukocytes, thrombocytes) (granulocytes <0.5x10_9/L/platelets < 20x10_9/L/reticulocytes <1.0% in presence of anemia) |
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Term
| Discuss the etiology & pathogenesis of aplastic anemia |
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Definition
Etiology:
-Idiopathic- pt. w/ no estab. history of chemical/drug exposure/ viral infection
-secondary (latrogenic)- drug related/ chemically related
-hereditary (constitutional)- genetic/predisposition to bone marrow failure
Pathogenesis: Immune response
-Antibodies direct against stem cell (what makes RBC, WBC, & Plt.)
-suppression of stem cell proliferation |
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Term
| Discuss the clinical & laboratory findings in aplastic anemia including clinical signs, peripheral blood, & bone marrow findings |
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Definition
Clinical- total bone marrow failure
Laboratory- pancytopenia (all cell lines decreased)
-RBC in peripheral blood typically appear normocytic/normochromic
Treatment-Immunosuppressive therapy
-if that doesn't work bone marrow transplant |
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