Hematology and Oncology Book

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ThalassemiaAka: Cooley's Anemia

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  1. Epidemiology
    1. Alpha thalassemia
      1. Asian patients (Incidence common)
      2. Black patients (Incidence 2%)
    2. Beta thalassemia
      1. Southern Italy and Mediterranean islands (0.1%)
      2. Central Africa
      3. Asia
      4. South Pacific
      5. India
  2. Types
    1. Alpha thalassemia
      1. Hydrops fetalis (Asian patients)
    2. Beta thalassemia
      1. Beta thalassemia major (Cooley's Anemia)
      2. Beta thalassemia minor
  3. Labs
    1. Complete Blood Count
      1. Hemoglobin or Hematocrit consistent with Anemia
      2. Mean Corpuscular Volume to Red Blood Cell Count ratio
        1. See Mentzer Index
        2. Ratio <13: Thalassemia
        3. Ratio >13: Iron Deficiency Anemia, Hemoglobinopathy
    2. Normal Iron study indices (no Iron Deficiency Anemia)
      1. Ferritin normal
      2. Total Iron Binding Capacity normal
      3. Serum Iron normal
    3. Variable Reticulocyte Index
      1. May see Reticulocytosis or Reticulocytopenia
    4. Peripheral Smear
      1. Hemolytic Anemia signs
      2. Red Blood Cell Inclusion bodies

Thalassemia (C0039730)

Definition (MSH)A group of hereditary hemolytic anemias in which there is decreased synthesis of one or more hemoglobin polypeptide chains. There are several genetic types with clinical pictures ranging from barely detectable hematologic abnormality to severe and fatal anemia.
Definition (CSP)heterogeneous group of hereditary hemolytic anemias which have in common a decreased rate of synthesis of one or more hemoglobin polypeptide chains.
ConceptsDisease or Syndrome (T047)
ICD9282.4, 282.49
EnglishHereditary leptocytosis, Thalassaemia, Thalassaemia syndrome, Thalassaemias, Thalassemia, Thalassemia syndrome, Thalassemias
Spanishsíndrome talasémico, sindrome talasemico, talasemia
CreditsDerived from the NIH UMLS (Unified Medical Language System)



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