Hereditary Pyropoikilocytosis Treatment & Management
- Author: Abdullah Kutlar, MD; Chief Editor: Emmanuel C Besa, MD more...
Treatment of hereditary pyropoikilocytosis includes transfusion of packed red blood cells and other supportive care. Supportive care encompasses intravenous fluids, oxygen, and monitoring in an acute care setting as determined by the needs of the individual patient. Further treatments such as medications or surgical procedures vary among patients, depending on their individual needs and other concurrent problems.
Splenectomy has been shown to improve anemia but has no effect on abnormal red blood cell morphology.
No dietary restrictions are necessary for people with hereditary pyropoikilocytosis. However, at some point, hemolysis and increased erythrocyte production by the bone marrow may necessitate folic acid supplements.
Activity is limited only in relation to the severity of the anemia and its effects on other organ systems.
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