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Hematology Chapter 13
Hypoproliferative Anemias
28
Biology
Graduate
11/12/2012

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Term
hypoproliferative anemia
Definition
Heterogeneous group of acquired and constitutional disorders in which there is a normocytic or macrocytic, normochromic anemia associated with chromic bone marrow hypocellularity.
Term
diagnostic criteria for aplastic anemia
Definition
requires Pancytopenia associated with hypocellular (less than normal amount of cells) bone marrow
Term
Epidemiology: aplastic anemia
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
Term
etiology: aplasic anemia
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.
Term
pathophysiology of aplastic anemia
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)
Term
Acquired aplastic anemia
Definition
usually idiopathic (50-70%), most common in adults, and is secondary to exposure
Term
Constitutional aplastic anemia
Definition
a birth defect, Fanconi’s anemia, Autosomal recessive, average survival is 25 years
Term
clinical characteristics of aplastic anemia
Definition
: insidious onset related to cytopenias, bleeding accompanied by petechial and fundal hemorrhages, anemia and infection
Term
laboratory characteristics of aplastic anemia
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
Term
environmental factors associated with the development of aplastic anemia
Definition
Drugs, chemicals, ionizing radiation, or biological agents
Term
Etiology pure red cell aplasia
Definition
: can be acquired (acute or chronic) or congenital (diamond-blackfan anemia)
Term
Bone marrow pure red cell aplasia
Definition
decrease of erythroid precursors, selective hypoproliferation of unipotent erythroid stem cell, no disturbance of granulocyte and platelet development
Term
Peripheral blood pure red cell aplasia
Definition
anemia
Term
Aplastic anemia peripheral blood findings
Definition
pancytopenia
Term
Pure red cell aplasia peripheral blood findings
Definition
decreased erythrocyte, decreased reticulocytes, normal leukocytes, normal platelets
Term
Anemia due to renal disease peripheral findings
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
Term
possible causes of aplastic anemia
Definition
Aplastic anemia can be acquired (due to exposures) or inherited (due to bone marrow defects)
Term
prognosis in aplastic anemia
Definition
Bone marrow transplant and immunosuppression
Term
Management of aplastic anemia
Definition
removal of a putative causative agent, withdrawal of drugs or removal of the patient from a hazardous environment
Term
treatment of aplastic anemia
Definition
: stem cell transplants, immunosuppressive therapy
Term
Aplastic anemia pancytopenia
Definition
always associated with bone marrow hypocellularity
Term
Pancytopenia can be caused
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
Term
Red cell aplasia is characterized by
Definition
decrease of erythroid precursors in the marrow
Term
Aplastic anemia is characterized by
Definition
a global pancytopenia in the blood and a hypocellular bone marrow.
Term
Diamond-Blackfans is characterized by
Definition
defect in the erythroid precursor cells
Term
Renal disease is characterized by
Definition
decreased hemoglobin and increased nitrogen urea in the blood
Term
Transient erythroblastopenia
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
Term
pathophysiology of anemia due to renal disease
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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