Pavord S R, Murphy P T, Mitchell V E
Department of Haematology, Leicester Royal Infirmary.
J Clin Pathol. 1996 Feb;49(2):181-2. doi: 10.1136/jcp.49.2.181.
A 58 year old man presented with a three year history of impotence, night sweats and ankle swelling. On examination, the patient fulfilled the diagnostic criteria for POEMS syndrome, but was unusual in that he also had underlying Waldenström's macroglobulinaemia with IgM kappa paraproteinaemia. The patient was treated with intermittent chlorambucil and made a good recovery. POEMS syndrome has been described in association with osteosclerotic myeloma and Castleman's disease. The paraprotein involved is usually IgG or IgA with lambda light chains. This case indicates that the presence of lambda light chains is not essential for the pathogenesis of POEMS syndrome. It also emphasises the diversity of plasma cell dyscrasias that can manifest as POEMS syndrome.
一名58岁男性有三年阳痿、盗汗和脚踝肿胀病史。检查时，该患者符合POEMS综合征的诊断标准，但不同寻常的是，他还患有潜在的华氏巨球蛋白血症伴IgM κ副蛋白血症。患者接受了间歇性苯丁酸氮芥治疗，恢复良好。POEMS综合征已被描述与骨硬化性骨髓瘤和Castleman病相关。所涉及的副蛋白通常为带有λ轻链的IgG或IgA。该病例表明λ轻链的存在对于POEMS综合征的发病机制并非必不可少。它还强调了可表现为POEMS综合征的浆细胞异常增生的多样性。
