Association between myeloid malignancies and acquired deficit in protein 4.1R:: A retrospective analysis of six patients

被引：15

作者：

Alanio-Brechot, Cecile ^{[1
]}

Schischmanoff, Pierre-Olivier ^{[2
,3
]}

Feneant-Thibault, Madeleine ^{[4
]}

Cynober, Therese ^{[1
]}

Tchernia, Gil ^{[1
]}

Delaunay, Jean ^{[1
]}

Garcon, Loic ^{[1
]}

机构：

[1] Hop Bicetre, Hematol Lab, Ctr Reference Maladies Constitutionelles Erythrop, AP HP, F-94275 Le Kremlin Bicetre, France

[2] Hop Avicenne, Biochim Lab, AP HP, F-93009 Bobigny, France

[3] Univ Paris 13, UFR SMBH, EA 3406, Bobigny, France

[4] Hop Bicetre, Biochim Lab, Ctr Reference Maladies Constitutionnelles Erythro, AP HP, Le Kremlin Bicetre, France

来源：

AMERICAN JOURNAL OF HEMATOLOGY | 2008年 / 83卷 / 04期

关键词：

D O I：

10.1002/ajh.21088

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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 abnormality in four out of six patients.

引用

页码：275 / 278

页数：4

共 19 条

[11] Elliptocytosis in myelodysplastic syndrome associated with translocation (1;5)(p10;q10) and deletion of 20q [J].