Table 3.

Management of Hb H disease with hemolytic crisis.

  1. Restore patient’s hemoglobin to 8–9 g/dL by red cell transfusion

    • - Provide filtered red blood cells or leucocyte depleted blood 5–12 mL/kg/dose depending on the patients’s clinical severity and levels of anemia

    • - A close monitoring on total body fluid and cardiovascular status is highly recommended.

    • -Serial Hb and Hct evaluation should be done at least daily since hemolysis could be continued as the cause has not been removed or properly treated.

  2. Give adequate hydration

    • - Intravenous fluid therapy should be provided to maintain circulation and withheld during transfusion support.

    • - The amount and rate should be carefully calculated to avoid possible heart failure from volume overload.

  3. Check blood electrolytes and provide appropriate correction

    • - Metabolic acidosis is usually observed but mostly resolved by transfusion support and fluid therapy. Only rare cases require alkali therapy.

  4. Try to control body temperature by various means

    • - Frequent tepid sponge

    • - Paracetamol 10–12 mg/kg every 4–6 hrs.

    • - The usage of NSAIDs in hemolytic crisis of Hb H has limited data.

  5. Identify the cause of infection/inflammation and provide appropriate treatment

    • - Blood and urine culture should be done.

    • - Empirical antibiotic with the coverage of gram-negative bacteria and/or encapsulated bacteria (depending on splenic condition) such as streptococcus, menigococcus and salmonella sps. should be promptly provided.

 
  1. Restore patient’s hemoglobin to 8–9 g/dL by red cell transfusion

    • - Provide filtered red blood cells or leucocyte depleted blood 5–12 mL/kg/dose depending on the patients’s clinical severity and levels of anemia

    • - A close monitoring on total body fluid and cardiovascular status is highly recommended.

    • -Serial Hb and Hct evaluation should be done at least daily since hemolysis could be continued as the cause has not been removed or properly treated.

  2. Give adequate hydration

    • - Intravenous fluid therapy should be provided to maintain circulation and withheld during transfusion support.

    • - The amount and rate should be carefully calculated to avoid possible heart failure from volume overload.

  3. Check blood electrolytes and provide appropriate correction

    • - Metabolic acidosis is usually observed but mostly resolved by transfusion support and fluid therapy. Only rare cases require alkali therapy.

  4. Try to control body temperature by various means

    • - Frequent tepid sponge

    • - Paracetamol 10–12 mg/kg every 4–6 hrs.

    • - The usage of NSAIDs in hemolytic crisis of Hb H has limited data.

  5. Identify the cause of infection/inflammation and provide appropriate treatment

    • - Blood and urine culture should be done.

    • - Empirical antibiotic with the coverage of gram-negative bacteria and/or encapsulated bacteria (depending on splenic condition) such as streptococcus, menigococcus and salmonella sps. should be promptly provided.

 

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