Hereditary Pyropoikilocytosis Treatment & Management

Updated: Aug 24, 2021
  • Author: Nellowe C Candelario, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Treatment

Approach Considerations

In the setting of  severe hemolytic anemia with hereditary pyropoikilocytosis, transfusion of packed red blood cells is necessary. Folate supplementation is also necessary in chronic hemolytic disease. In the acute care setting, supportive measures (eg, intravenous fluids, oxygen, monitoring of blood counts) are provided according to the needs of the individual patient.

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Medical Care

No specific medications are used to treat patients with hereditary pyropoikilocytosis. The need for treatment with medications is patient-specific and based on individual complications. Folic acid is often used to prevent folic acid deficiency, which may occur as a result of increased erythropoiesis. Iron chelation therapy may be necessary in patients who develop significant iron overload from red blood cell transfusions.

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Surgical Care

The spleen is the primary site of red cell sequestration and contribute to the severity of anemia in patients with HPP. Splenectomy has been advocated by some experts for severe hemolytic anemia refractory to or complicated by chronic blood trasnfusion. Splenectomy has been reported to improve the severity of anemia, symptoms and incidence of gallstones. [10]

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Long-Term Monitoring

Provide routine outpatient care and monitor hemoglobin levels if symptoms occur. Maintain high suspicion for early development of gallbladder disease. Iron chelation therapy may be required to prevent irreversible end-organ damage due to transfusion-induced hemosiderosis. Monitor the number of transfusions performed and the iron status of the patient to determine if and when this therapy is needed.

Routine vaccination against pneumococcal and meningococcal disease and Haemophilus influenzae prior to splenectomy is important in preventing morbidity from these pathogens.

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