Richard C, Cuadrado M A, Prieto M, Batlle J, López Fernández M F, Rodriguez Salazar M L, Bello C, Recio M, Santoro T, Gomez Casares M T
Department of Hematology, Faculty of Medicine, National Hospital Marqués de Valdecilla, Santander, Spain.
Am J Hematol. 1990 Oct;35(2):114-7. doi: 10.1002/ajh.2830350210.
A case of acquired von Willebrand disease (AvWD) associated with an IgA lambda multiple myeloma is reported. No form of inhibitor could be detected. SDS-agarose gel electrophoresis patterns of von Willebrand factor (vWF) both in plasma and platelet lysates were normal but a decrease in all-sized multimers with a type IA pattern was seen. After 1-deamino-8-D arginine vasopressin (DDAVP) infusion, vWF multimers larger than those seen in the resting state appeared in patient plasma, which were progressively cleared. Indirect immunofluorescence studies with a monoclonal antibody to vWF showed that vWF was selectively absorbed into myelomatous cells. This is the first case of AvWD associated with multiple myeloma resulting from the selective absorption of vWF into abnormal plasma cells. This feature established a new pathophysiological mechanism of AvWD in multiple myeloma and probably in other lymphoproliferative diseases.
报告了一例与IgA λ型多发性骨髓瘤相关的获得性血管性血友病（AvWD）病例。未检测到任何形式的抑制剂。血浆和血小板裂解物中血管性血友病因子（vWF）的SDS-琼脂糖凝胶电泳图谱正常，但可见所有大小多聚体均减少，呈IA型模式。输注1-去氨基-8-D-精氨酸血管加压素（DDAVP）后，患者血浆中出现了比静息状态下更大的vWF多聚体，这些多聚体逐渐被清除。用抗vWF单克隆抗体进行的间接免疫荧光研究表明，vWF被选择性地吸收到骨髓瘤细胞中。这是首例因vWF被选择性吸收到异常浆细胞中而导致的与多发性骨髓瘤相关的AvWD病例。这一特征确立了多发性骨髓瘤以及可能其他淋巴增殖性疾病中AvWD的一种新的病理生理机制。
