Term
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Definition
Heterogeneous group of acquired and constitutional disorders in which there is a normocytic or macrocytic, normochromic anemia associated with chromic bone marrow hypocellularity. |
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Term
diagnostic criteria for aplastic anemia |
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Definition
requires Pancytopenia associated with hypocellular (less than normal amount of cells) bone marrow |
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Term
Epidemiology: aplastic anemia |
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Definition
most commonly seen in 2-5 year olds (due to inherited forms), 15-25 year olds (due to acquired forms), and adults over 60. There are more cases in Asia than in western countries |
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Term
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Definition
acquired forms due to environmental exposures or idiopathic, constitutional forms due to chronic failure of the bone marrow due to inherited abnormality which may be present at birth. |
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Term
pathophysiology of aplastic anemia |
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Definition
Due to immunologic suppression of hematopoiesis (Tc cells inhibit stem cells or Epstein Barr viral infections), deficient, defective stem cells (acquired or inherited), defective microenvironment of bone marrow (rare), and deficiency of hematopoietic growth factors (no evidence) |
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Term
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Definition
usually idiopathic (50-70%), most common in adults, and is secondary to exposure |
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Term
Constitutional aplastic anemia |
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Definition
a birth defect, Fanconi’s anemia, Autosomal recessive, average survival is 25 years |
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Term
clinical characteristics of aplastic anemia |
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Definition
: insidious onset related to cytopenias, bleeding accompanied by petechial and fundal hemorrhages, anemia and infection |
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Term
laboratory characteristics of aplastic anemia |
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Definition
peripheral blood shows Pancytopenia, hemoglobin is below 7.0 g/dL, erythrocytes appear normocytic and normochromic, bone marrow shows hypocellular with more than 70% fat |
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Term
environmental factors associated with the development of aplastic anemia |
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Definition
Drugs, chemicals, ionizing radiation, or biological agents |
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Term
Etiology pure red cell aplasia |
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Definition
: can be acquired (acute or chronic) or congenital (diamond-blackfan anemia) |
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Term
Bone marrow pure red cell aplasia |
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Definition
decrease of erythroid precursors, selective hypoproliferation of unipotent erythroid stem cell, no disturbance of granulocyte and platelet development |
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Term
Peripheral blood pure red cell aplasia |
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Definition
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Term
Aplastic anemia peripheral blood findings |
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Definition
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Term
Pure red cell aplasia peripheral blood findings |
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Definition
decreased erythrocyte, decreased reticulocytes, normal leukocytes, normal platelets |
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Term
Anemia due to renal disease peripheral findings |
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Definition
normochromic normocytic anemia, moderate aniso (some microcytes), hemoglobin levels low (5.0-8.0 g/dL), RPI 1 (low), moderate to severe Poikilocytosis (Echinocytes, Schistocytes, sphereocytes, acanthocytes, and nRBCs) WBCs and platelets are normal |
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Term
possible causes of aplastic anemia |
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Definition
Aplastic anemia can be acquired (due to exposures) or inherited (due to bone marrow defects) |
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Term
prognosis in aplastic anemia |
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Definition
Bone marrow transplant and immunosuppression |
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Term
Management of aplastic anemia |
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Definition
removal of a putative causative agent, withdrawal of drugs or removal of the patient from a hazardous environment |
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Term
treatment of aplastic anemia |
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Definition
: stem cell transplants, immunosuppressive therapy |
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Term
Aplastic anemia pancytopenia |
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Definition
always associated with bone marrow hypocellularity |
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Term
Pancytopenia can be caused |
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Definition
myelophthisic anemia (infiltration of bone marrow by fibrotic, granulomatous, or neoplastic cells, immature cells released to the blood. Myelodysplastic syndrome is a disorder that tends to terminate in acute leukemia; bone marrow cellularity appears normal or increased. Hypersplenism is increased sequestration by spleen and B12 and folate deficiency |
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Term
Red cell aplasia is characterized by |
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Definition
decrease of erythroid precursors in the marrow |
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Term
Aplastic anemia is characterized by |
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Definition
a global pancytopenia in the blood and a hypocellular bone marrow. |
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Term
Diamond-Blackfans is characterized by |
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Definition
defect in the erythroid precursor cells |
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Term
Renal disease is characterized by |
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Definition
decreased hemoglobin and increased nitrogen urea in the blood |
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Term
Transient erythroblastopenia |
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Definition
temporary suppression of Erythropoiesis that frequently occurs after a viral infection in otherwise normal children. Fetal characteristics of erythrocytes seen in DBA are not found in TEC. TEC is thought to be virus-associated or antibody mediated |
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Term
pathophysiology of anemia due to renal disease |
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Definition
Decreased EPO production by kidneys, shortened RBC survival, blood loss due to thrombocytopenia, dialysis (mechanical disturbance, iron and folate are dialyzable) |
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