Lagueny A, Bouillot S, Vital C, Ferrer X, Larrieu J-M, Vital A
Service de Neurologie, Hôpital Haut-Lévêque, CHU Bordeaux, Pessac, France.
Rev Neurol (Paris). 2004 Mar;160(3):285-95. doi: 10.1016/s0035-3787(04)70903-0.
POEMS is an acronym for polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes. POEMS syndrome is also called Crow-Fukase syndrome, chiefly in Japan. The 5 above mentioned features are not always present at the first examination. The minimal criteria to establish the diagnosis are the presence of a demyelinating and axonal polyneuropathy associated with an IgA or IgG monoclonal gammopathy, the light chain being almost always lambda, and at least 2 of the 8 other features: sclerosing plasmocytoma, endocrinopathy, skin changes, organomegaly, Castleman's disease, anasarca, papillary edema or thrombocytosis. Among these features, only cutaneous glomeruloid angioma are specific. Ultrastructural identification of uncompacted myelin lamellae on the peripheral nerve biopsy is also a strong argument in favor of the diagnosis. An associated "osteosclerotic" bone lesion must be carefully searched, because its treatment may improve the other features of the syndrome, especially the neuropathy. Cytokines and the vascular growth endothelial factor might play a role in the pathogenesis of this rare multisystemic disorder.
POEMS是多发性神经病、器官肿大、内分泌病、M蛋白和皮肤改变的英文首字母缩写。POEMS综合征在日本主要被称为Crow-Fukase综合征。上述5种特征在初次检查时并不总是都存在。确立诊断的最低标准是存在与IgA或IgG单克隆丙种球蛋白病相关的脱髓鞘和轴索性多发性神经病，轻链几乎总是λ链，以及8种其他特征中的至少2种：硬化性浆细胞瘤、内分泌病、皮肤改变、器官肿大、Castleman病、全身性水肿、视乳头水肿或血小板增多症。在这些特征中，只有皮肤肾小球样血管瘤具有特异性。周围神经活检中未紧密排列的髓鞘板层的超微结构鉴定也是支持该诊断的有力依据。必须仔细查找相关的“骨硬化性”骨病变，因为其治疗可能改善该综合征的其他特征，尤其是神经病。细胞因子和血管生长内皮因子可能在这种罕见的多系统疾病的发病机制中起作用。
