TY - JOUR
T1 - A case of atypical POEMS syndrome without polyneuropathy
AU - Morizane, Ryuji
AU - Sasamura, Hiroyuki
AU - Minakuchi, Hitoshi
AU - Takae, Yujiro
AU - Kikuchi, Haruhito
AU - Yoshiya, Naomi
AU - Hashiguchi, Akinori
AU - Konishi, Konosuke
AU - Okamoto, Shinichiro
AU - Itoh, Hiroshi
PY - 2008/5
Y1 - 2008/5
N2 - 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.
AB - 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.
KW - Neuropathy
KW - Osteosclerotic myeloma
KW - POEMS syndrome
UR - http://www.scopus.com/inward/record.url?scp=41849097753&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=41849097753&partnerID=8YFLogxK
U2 - 10.1111/j.1600-0609.2008.01045.x
DO - 10.1111/j.1600-0609.2008.01045.x
M3 - Article
C2 - 18284621
AN - SCOPUS:41849097753
SN - 0902-4441
VL - 80
SP - 452
EP - 455
JO - European Journal of Haematology
JF - European Journal of Haematology
IS - 5
ER -