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Abnormal platelet von willebrand factor (vWF) as a marker of abnormal function in megakaryocytic dysplasia
Author(s) -
de Cataldo F.,
Baudo F.,
Redaelli R.,
Corno A. R.
Publication year - 1995
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.2830480303
Subject(s) - von willebrand factor , dysplasia , platelet , abnormality , haematopoiesis , megakaryocyte , medicine , biology , genetics , stem cell , psychiatry
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 α 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. ©1995 Wiley‐Liss, Inc.