de Cataldo F, Baudo F, Redaelli R, Corno A R
Department of Hematology, Ospedale Niguarda Cà Granda, Milan, Italy.
Am J Hematol. 1995 Mar;48(3):155-7. doi: 10.1002/ajh.2830480303.
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 explantation of the abnormality.
骨髓增生异常综合征（MDS）是造血系统的肿瘤性疾病；多系受累也可通过特定的细胞功能障碍得到证实。血管性血友病因子（vWF）在巨核细胞（MK）中合成和加工，储存在血小板的α颗粒中。研究了18例MDS患者和4例恶性贫血（PA）患者的血小板vWF多聚体模式，以调查巨核细胞发育异常时vWF的加工是否异常。10/18例MDS患者和4/4例PA患者观察到异常的多聚体模式。这种特定蛋白质的异常是基本疾病的离散表现，当造血正常化时是可逆的。虽然这些数据无法得出任何结论，但异常合成可能是异常的解释。
