Hyman B T, Westrick M A
Arch Intern Med. 1986 May;146(5):993-4. doi: 10.1001/archinte.146.5.993.
A patient with the syndrome of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) had multiple myeloma and IgA-kappa monoclonal gammopathy. To our knowledge, of over 50 cases in the literature, this is the only instance of kappa light chains noted in a patient with the complete syndrome. Sural nerve biopsy revealed increased immunofluorescence for IgA and kappa chains. Furthermore, this patient had a hemorrhagic diathesis characterized by a prolonged bleeding time and a prolonged thrombin time due to a qualitative platelet dysfunction and an apparent inhibition of fibrin monomer polymerization. In none of the other cases of POEMS syndrome was a coagulopathy reported. A short course of plasmapheresis improved the bleeding time but did not alter the polyneuropathy.
一名患有多神经病、器官肿大、内分泌病、M蛋白和皮肤改变综合征(POEMS)的患者患有多发性骨髓瘤和IgA-κ单克隆丙种球蛋白病。据我们所知,在文献报道的50多例病例中,这是唯一一例在患有完整综合征的患者中发现κ轻链的情况。腓肠神经活检显示IgA和κ链的免疫荧光增强。此外,该患者存在出血素质,其特征为出血时间延长和凝血酶时间延长,原因是血小板功能定性异常以及纤维蛋白单体聚合明显受抑制。在其他POEMS综合征病例中均未报告有凝血病。短期血浆置换改善了出血时间,但并未改变多神经病的症状。