Definition and classification

  • Define hereditary hemolytic anemia. hl
  • Please tell the name of some hereditary hemolytic anemia. Hl
    • What are more common in this subcontinent? Hl
  • What is the normal lifespan of RBC? Hl
  • Tell the name of normal hemoglobins with globin chains. Hl
  • Please tell the percentage of different hemoglobins at birth. Hl
  • What is hereditary disease? Hl
  • What is congenital disease? hl
  • What is thalassemia? A, hl
  • What is hypertransfusion and supertransfusion? Hl
  • What is iron overload? Hl
  • Iron overload anemia occurs in in spite of anemia in which condition? Hl
    • What condition develop from iron overload? hl
  • Define hemosiderosis and hemochromatosis? Hl


  • Q

Etiology and Pathophysiology

  • What is the mode of inheritance of thalassemia? Hl
  • What are the causes of the microcytic hypochromic blood picture? A
  • When anemia develops in a patient with thalassemia major? A, hl
  • Why is there jaundice in thalassemia major? Hl
  • What are the cause of pain abdomen in thalassemia major? hl
  • What are the target organs of hemochromatosis? Hl
  • What are the infectious agents for which splenectomized patients are susceptible? hl

Clinical manifestations

  • What are the triad of hemolytic anemia? hl
  • What are the presentations of thalassemia minor? A
  • Please tell the clinical features of thalassemia. H62
  • What are the features of hypersplenism? Hl


  • Q


  • What investigations should be done for hereditary hemolytic anemia? A, hl
  • How can you diagnose thalassemia by investigation? H64
  • What are the findings in PBF? hl
  • Mention one single investigation which is helpful for your diagnosis. A
  • What another hematological investigation is helpful for your diagnosis? A
  • How will you confirm your diagnosis? A, hl
  • What are the findings in Hb-electrophoresis in b-thalassemia? A
  • What are the findings in Hb-electrophoresis in thalassemia major? hl
  • What are the findings in Hb-electrophoresis in thalassemia minor? hl
  • What are the radiological findings in skull in b-thalassemia major? A, hl
  • What are the findings in iron profile? hl


  • What hereditary hemolytic anemia do you consider? Hl
  • Why do you consider this diagnosis? hl
  • What are your differential diagnoses? A
  • Why do you not consider chronic malaria, kala-azar and chronic liver disease as differential diagnosis?
  • A 12-year-old boy presents with tiredness with history of multiple blood transfusion. On examination patient is anemic, have jaundice and splenomegaly. What is your diagnosis? H52 (Hereditary hemolytic anemia.)
  • What are the diagnostic criteria of hemolytic anemia? A, hl
  • If the patient develops severe abdominal pain, what is the likely cause? A
  • How can it be diagnosed before birth? A
  • What is the differential diagnosis of thalassemia minor? A
  • A 25-year-old man presents with mild anemia with microcytosis and hypochromia on PBF but not responding to adequate iron therapy. What are you thinking? H5
    • What investigation would you suggest? H6


  • Please tell the treatment of thalassemia? Hl
  • How to treat b-thalassemia major? A
  • What are the indications of splenectomy in thalassemia? Hl
  • What is the prerequisite for splenectomy? Hl
  • What are the components of counseling in thalassemia? Hl
  • How frequently is blood transfusion needed? Hl
  • Folic acid supplementation should be given in thalassemia, why? Hl
  • What are the indications of blood transfusion in thalassemia? H74
  • How can you treat iron overload? hl


  • What are the complications of thalassemia major? hl
  • Please mention the complications of transfusion in thalassemia? Hl
  • What are the complications of repeated blood transfusion? A
    • How hemosiderosis can be prevented? A
  • What are the causes of death in thalassemia? Hl



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