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
Epidemiology
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
Examinations
Investigations
- 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
Diagnosis
- 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
Treatment
- 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
Complications
- 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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