title: Pathological findings in three non-Japanese patients with the POEMS syndrome.
abstract: The pathological features of three European patients with plasma cell dyscrasia, osteosclerosis and a multisystem disorder, most frequent in Japan, that includes polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS syndrome), are reported. The material was obtained from biopsies (peroneal nerve, lymph node) and general autopsy, including hypophysis, in one case. The most salient findings were: peripheral nerve lesions, including both segmental demyelination and axonal degeneration, with so-called uncompacted myelin lamellae (UML); angiofollicular lymph node hyperplasia (AFLNH); and non inflammatory vascular changes. Though not specific, it appears that UML and AFLNH may be contributive findings in atypical cases of POEMS syndrome (incomplete forms, lack of underlying malignant plasma cell dyscrasia or circulating monoclonal immunoglobulin). Among the various autopsy findings we emphasize the skin thickening which was secondary to a hyaline sclerosis of the papillary dermis, and the presence in adenohypophysis of numerous cells showing positive reactions with the anti-alpha MSH antibody. Though immunological, vascular and hormonal disturbances have been implicated at the origin of several manifestations of the disorder, the pathogenesis of the POEMS syndrome remains obscure.
authors.affiliation: Département de Pathologie, Hôpital Henri Mondor, Créteil, France.
authors.forename: R
authors.initials: R
authors.lastname: Gherardi
authors.forename: M
authors.initials: M
authors.lastname: Baudrimont
authors.forename: M
authors.initials: M
authors.lastname: Kujas
authors.forename: D
authors.initials: D
authors.lastname: Malapert
authors.forename: F
authors.initials: F
authors.lastname: Lange
authors.forename: F
authors.initials: F
authors.lastname: Gray
authors.forename: J
authors.initials: J
authors.lastname: Poirier
mesh_terms: D000328:Adult; D000368:Aged; D001369:Axons / Q000473:pathology / Q000648:ultrastructure; D001798:Blood Proteins / Q000378:metabolism; D001853:Bone Marrow / Q000473:pathology; D004487:Edema; D004700:Endocrine System Diseases / Q000473:pathology*; D005260:Female; D006529:Hepatomegaly; D006801:Humans; D007136:Immunoglobulins; D007150:Immunohistochemistry; D008198:Lymph Nodes / Q000473:pathology; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D009194:Myeloma Proteins / Q000378:metabolism; D009412:Nerve Fibers / Q000473:pathology / Q000648:ultrastructure; D010265:Paraproteinemias / Q000473:pathology*; D010523:Peripheral Nervous System Diseases / Q000473:pathology*; D012867:Skin / Q000473:pathology*; D013163:Splenomegaly; D013577:Syndrome
chemical_list: D001798:Blood Proteins; D007136:Immunoglobulins; C009823:M-proteins (Myeloma); D009194:Myeloma Proteins