Konno M, Yoshioka A, Takase T, Imai T
Department of Pediatrics, Sapporo Kosei General Hospital, Japan.
Acta Paediatr Jpn. 1995 Feb;37(1):97-100. doi: 10.1111/j.1442-200x.1995.tb03697.x.
We report on a Japanese girl with Upshaw-Schulman syndrome, a congenital disorder characterized by recurrent thrombocytopenia, microangiopathic hemolytic anemia, proteinuria and hematuria that can be transiently improved by the transfusion of plasma or various plasma components. Unusually Large von Willebrand Factor (ULvWF) multimers were found during both relapse and remission phases. Serial plasma levels of 6-keto-prostaglandin F1 alpha (PGF1 alpha), the stable metabolite of prostacyclin (PGI2), were low at relapse. When the patient was treated with continuous PGI2 infusion, the microangiopathic hemolytic process gradually subsided within 10 days. These results suggest that PGI2 may be partly involved in the pathogenesis of this congenital disorder.
我们报告了一名患有Upshaw-Schulman综合征的日本女孩，这是一种先天性疾病，其特征为反复血小板减少、微血管病性溶血性贫血、蛋白尿和血尿，输注血浆或各种血浆成分可使其暂时改善。在复发期和缓解期均发现了异常大的血管性血友病因子（ULvWF）多聚体。在复发时，前列环素（PGI2）的稳定代谢产物6-酮-前列腺素F1α（PGF1α）的系列血浆水平较低。当该患者接受持续PGI2输注治疗时，微血管病性溶血过程在10天内逐渐消退。这些结果表明，PGI2可能部分参与了这种先天性疾病的发病机制。
