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Association between myeloid malignancies and acquired deficit in protein 4.1R: A retrospective analysis of six patients
Author(s) -
AlanioBréchot Cécile,
Schischmanoff PierreOlivier,
FénéantThibault Madeleine,
Cynober Thérèse,
Tchernia Gil,
Delaunay Jean,
Garçon Loïc
Publication year - 2008
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.21088
Subject(s) - medicine , myelodysplastic syndromes , myeloid , abnormality , immunology , oncology , bone marrow , psychiatry
Constitutional deficit in the erythroid protein 4.1 (4.1R), a structural component of the erythrocyte membrane, is implicated in hereditary elliptocytosis. Acquired deficit in protein 4.1R have been rarely described in myelodysplastic syndromes. Here, we report a series of six patients presenting a myelodysplastic or a myeloproliferative disease in association with an elliptocytosis curve on osmotic gradient ektacytometry and a significant decrease in protein 4.1R level. We confirm that deficit in protein 4.1R is recurrent in myeloid malignancies and should be particularly investigated when deletion del (20q) is present, since we found this chromosomal abnormalityin four out of six patients. Am. J. Hematol., 2008. © 2007 Wiley‐Liss, Inc.