HEMPAS

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HEMPAS

Hereditary erythrocytic multinuclearity with positive acidified serum lysis, Congenital dyserythropoietic anemia, type II An AR condition characterized by an IgM autoantibody against the RBC's i antigen–'anti-HEMPAS', an antigen is present in¹⁄₃ of normal sera Clinical Mild or subclinical congenital dyserythropoietic anemia Lab Normocytic aniso-poikilocytosis and multi-nucleated erythroblasts; ↑ agglutinability of serum with anti-i antibodies Treatment None; splenectomy is partially beneficial. See CDA.

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