HEMPAS


Also found in: Acronyms.

HEMPAS

Acronym for hereditary erythroblastic multinuclearity associated with positive acidified serum. See: HEMPAS cells.

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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References in periodicals archive ?
Congenital dyserythropoietic anaemia type II (HEMPAS) and its molecular basis.
Incompletely processed N-glycans of serum glycoproteins in congenital dyserythropoeitic anaemia type II (HEMPAS).
[4] Nonstandard abbreviations: ER, endoplasmic reticulum; GalNAc, N-acetylgalactosamine; GlcNAc, N-acetylglucosamine; SA, sialic acid; Con A, concanavalin A; AGP, [[alpha].sub.1], acid glycoprotein; CDG, congenital disorders of glycosylation; TRF, transferrin; LAD, leukocyte adhesion deficiency; HEMPAS, hereditary erythroblastic multinuclearity with a positive acidified serum test; WAS, Wiskott-Aldrich syndrome; ALP, alkaline phosphatase; AFP, [alpha]-fetoprotein; and LCA, Lens culinaris agglutinin.
Protein glycosylation and diseases: blood and urinary oligosaccharides as markers for diagnosis and therapeutic monitoring

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