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Hereditary spherocytosis

  1. Autosomal dominantQ
  2. The most common molecular defects are abnormalities of spectrin or ankyrin which are major component of cytoskeleton responsible for RBC shape. Q
  3. The decreased deformability of the spherocytic RBC impairs the cell passage from the splenic cord to the splenic sinuses and the spherocytic RBC are destroyed prematurely in the spleen.         

Clinical features-

  1. May cause hemolytic disease of new born and may present with anemia and hyperbilirubinemia sufficiently severe to require phototherapy and exchange.
  2. Spleen may usually enlarge after infancy
  3. Pigmentary gall stones may form as early as age 4-5 year. 

Lab findings

  1. Hb 6-lOgm%                
  2. Retic count 6-20%        
  3. MCV-normal
  4. MCHC- increased e. PB                
  5. Spherocytes                
  6. Osmotic fragility increasedQ
  7. Coombs test- negativeQ 


  1. Splenectomy when Q
    1. Hb ≥ 10gm%
    2. Retic > 10%
  2. Vaccine for encapsulated organism such as Pneumococcus, Meningococcus and Haemophilus influenzae type b should be administered before splenectomy.
  3. Oral penicillin prophylaxis given.

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