(Crow-Fukase Syndrome; Takatsuki Disease; PEP Syndrome)
POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes) is a nonautoimmune polyglandular deficiency syndrome.
POEMS syndrome is probably caused by circulating immunoglobulins caused by a plasma cell dyscrasia. Circulating cytokines (IL-1-β, IL-6), vascular endothelial growth factor, and tumor necrosis factor-alpha are also increased.
Patients may have the following:
Other symptoms and signs may include edema, ascites, pleural effusion, papilledema, and fever.
Like other syndromes of undefined pathophysiology, POEMS syndrome is diagnosed based on the constellation of symptoms and signs. Criteria include the presence of polyneuropathy and monoclonal paraproteinemia plus any 2 of the other manifestations of the disorder.
Treatment consists of radiation therapy or chemotherapy sometimes followed by autologous hematopoietic stem cell transplantation. Five-year survival is about 60%.