ABNORMAL PLATELET VON-WILLEBRAND-FACTOR (VWF) AS A MARKER OF ABNORMAL FUNCTION IN MEGAKARYOCYTIC DYSPLASIA

被引:4
|
作者
DECATALDO, F
BAUDO, F
REDAELLI, R
CORNO, AR
机构
[1] Department of Hematology, Ospedale Niguarda Ca Granda, Milan
关键词
PLATELET VWF; MYELODYSPLASTIC SYNDROMES; PERNICIOUS ANEMIA;
D O I
10.1002/ajh.2830480303
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
The myelodysplastic syndromes (MDS) are neoplastic disorders of the hemopoietic system; multilineage involvement is also evidenced by specific cellular dysfunctions. The von Willebrand factor (VWF), synthesized and processed in the megakaryocytes (MK), is stored in the alpha granules of the platelets., The platelet vWF multimeric pattern was studied in 18 patients with MDS, and in 4 with pernicious anemia (PA), to investigate whether the processing of vWF is abnormal in the megakaryocytic dysplasia. An abnormal multimeric pattern was observed in 10/18 MDS and 4/4 PA patients. The abnormality of this specific protein is the discrete expression of the basic disorder, and is reversible when hemopoiesis is normalized, Although the data do not allow any conclusion, abnormal synthesis is the likely explanation of the abnormality. (C) 1995 Wiley-Liss, Inc.
引用
收藏
页码:155 / 157
页数:3
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