POEMS syndrome
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(Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal, Skin)
(Crow-Fukase syndrome, Takatsuki syndrome, PEP syndrome (acronym for Polyneuropathy Endocrinopathy Plasma cell dyscrasia), Shimpo syndrome or disease).
Paraneoplastic syndrome of unknown etiology appearing in adulthood and combining a monoclonal dysglobulinemia, a peripheral symmetric, sometimes painful polyneuropathy (initially, sensory and later motor), a plasma protein anomaly (similar to multiple myeloma gammopathy), an organomegaly (liver, spleen, lymph nodes), a polyendocrinopathy (diabetes, hypogonadism) and skin alterations (dark colouring, hypertrichosis). It is probably a chronic inflammatory disease with high cytokines blood levels, including VEGF (vascular endothelial growth factor). One can also observe: osseous sclerotic lesions (sometimes associated with lytic lesions), papilledema, pleural effusion, ascites, thrombocytosis, vascular thrombosis. Pulmonary arterial hypertension has been reported. A quarter of patients affected by Castleman disease develops POEMS syndrome in adulthood.
This syndrome is often confused at its onset with chronic inflammatory demyelinating polyneuropathy.
Treatment: corticosteroids, local radiotherapy, chemotherapy; sometimes bone marrow transplantation.
Anesthetic implications :
interactions with chemotherapy and steroids; check for the absence of pulmonary hypertension; check endocrine functions
References :
- Dispenzieri A, Kyle RA, Lacy MQ et al.
POEMS syndrome : definitions and long-term outcome.
Blood 2003; 101: 2496-506.
Updated: October 2018