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20 Cards in this Set
- Front
- Back
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Aplastic Anemia
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- immune response may influence hematopoiesis
- reduced growth or production of blood cells - proinflammatory cytokines, TNF-alpha, and interferon-gama are involved - most prevelant is idiopathic - secondary to etiological that are chemical or drug (iatrogenic) * iatrogenic when the transient marrow failue follows cytotoxic chemo or radiation therapy |
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Pathophysiology of aplastic anemia
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- caused by immune process if sudden
- includes autoantibodies directed at stem cells or via T-lymphs that suppress stem cells - decrease activity of any precursor cell in marrow - humoral and cellular stimulators absent |
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Clinical features of aplastic anemia
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- total bone marrow failure with a reduced level of RBC, WBC, and plts
- acute or chronic - bleeding, infection, and anemia |
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Lab findings of aplastic anemia
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- pancytopenia usually RBCs
- increase serum iron - severe if 2/3 fall below critical levels - hypocelluar marrow - normachromic and normacytic |
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Fanconi's Anemia
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- congenital form of aplastic anemia
- accounts for 25-30% of childhood aplastic anemia - autosomal recessive - chromatid breaks, gaps, rearrangement, reduplications, and exchanges - 2x more in males |
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Lab findings of Fanconi's Anemia
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- progressive pancytopenia
- Hgb: 5-6g/dL - susceptible to an immunce cytokine (genetic defect) - hypersensitivity to the clastogenic effect of DNA cross-linking agents such as diepoxybutane |
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Pure Red Cell Aplasia
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- decrease in erythropoiesis
- immune suppression is said to be a factor b/c pts. respond to steroid therapy - pts have antibodies directed at erythroid precursors and lymphs capable of inhibiting erythropoiesis - serum erythropoietin increased - malnutrition and malignancies accounts for 1/2 of cases |
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Diamond-Blackfan Syndrome
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- congenital hypoplastic anemia
- a rare congenital form of red cell aplasia - defective stem cell (BFU-E) - refractory anemia w/o leukopenia or thrombocytopenia - early infancy - spontaneous remission - progenitor cells are unreponsive to EPO |
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Lab Data of Diamond-Blackfan Syndrome
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- normochromic
- slight macrocyte - decrease retic count - reduction of erythroid line - fetal Hg increase (5-25%) |
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Transient Erythroblastopenia of Childhood
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- occurs in previously healthy kids
- history of viral infection - humoral inhibition of RBC progenitors or decrease stem cells - mod to severe normocytic anemia w/ severe reticulocytopenia |
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Acquired aplastic anemia may be caused by:
A.) benzene or benzene derivatives B.) ionizing radiation and vitamin B12 C.) purine or pyrimidine analogues D.) all of the above |
D.) all of the above
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The sudden appearance of aplastic anemia or pure red cell aplasia is often caused by:
A.) a hemolytic process B.) an immune process C.) acute leukemias D.) chronic leukemias |
B.) an immune process
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Aplastic anemia can occur years before a diagnosis of____is made.
A.) paroxysmal nocturnal hemoglobinuria B.) myelodysplasia C.) acute myelogenous leukemia D.) all of the above |
D.) all of the above
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If a patient with aplastic anemia is referred to as exhibiting pancytopenia, which cell lines are affected?
A.) Erythrocytes B.) Leukocytes C.) Thrombocytes D.) All of the above |
D.) all of the above
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A subset of Fanconi's anemia
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familial aplastic anemia
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A rare congenital form of red cell aplasia
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Diamond-Blackfan syndrome
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Is characterized by selective failure of red blood cell production
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Pure red cell anemia
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The best-described congenital form of aplastic anemia
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Fanconi's anemia
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Hematopoietic cell targets in aplastic anemia are affected by:
A.) activated cytotoxic T lymphocytes B.) activation of the Fas receptor C.) direct cell-cell interactions between lymphocytes and target cells D.) all of the above |
D.) all of the above
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Fanconi's anemia is associated with an abnormal genes located on chromosomes____,____.
A.) 9, 20 B.) 5, 22 C.) 9, 12 D.) 8, 23 |
A.) 9, 20
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