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A case of atypical POEMS syndrome without polyneuropathy
Author(s) -
Morizane Ryuji,
Sasamura Hiroyuki,
Minakuchi Hitoshi,
Takae Yujiro,
Kikuchi Haruhito,
Yoshiya Naomi,
Hashiguchi Akinori,
Konishi Konosuke,
Okamoto Shinichiro,
Itoh Hiroshi
Publication year - 2008
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/j.1600-0609.2008.01045.x
Subject(s) - poems syndrome , organomegaly , polyneuropathy , medicine , pathology , dermatology , polyradiculoneuropathy , pediatrics , guillain barre syndrome
POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, M‐protein, Skin changes) syndrome is a rare hematological disease associated with overproduction of pro‐inflammatory cytokines. Under the current nomenclature and diagnostic criteria for POEMS syndrome, the presence of characteristic polyneuropathy is required for diagnosis. We report a 43‐year‐old Japanese woman with organomegaly, endocrinopathy, M‐protein, skin lesions, as well as typical renal lesions and sclerotic bone lesions. Of note, neurological examinations and peripheral nerve conduction tests were normal in this patient. In view of the overwhelming number of otherwise characteristic signs and symptoms, we made a provisional diagnosis of ‘atypical POEMS syndrome without polyneuropathy’. If further similar cases are reported in the future, reconsideration of the nomenclature and/or diagnostic criteria for POEMS syndrome may be required.