POEMS Syndrome

POEMS syndrome (Crow-Fukase syndrome) is a paraneoplastic syndrome associated with plasma-cell dyscrasia (osteosclerotic myeloma/Castleman disease). The acronym stands for Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin changes. Other features: sclerotic bone lesions, papilledema, thrombocytosis, edema/ascites, pulmonary hypertension. Elevated VEGF is central to pathogenesis.

Common Symptoms

Progressive symmetric polyneuropathy; hepatosplenomegaly/lymphadenopathy; endocrinopathy (hypothyroidism, diabetes, gynecomastia); M-protein; skin hyperpigmentation/hypertrichosis; edema; papilledema

Causes and Pathogenesis

Plasma-cell clone overproduces VEGF and cytokines, driving neovascularization, increased vascular permeability, neuropathy, organomegaly and endocrinopathy.

Treatment

Local radiation/surgery for solitary osteosclerotic lesion; lenalidomide/dexamethasone; autologous stem-cell transplant; bortezomib

Preventive Measures

Early diagnosis; treat underlying plasma-cell disorder; VEGF lowering.

Department to Visit

It is recommended to visit the Hematology department; an appointment can be booked on this platform before the visit.

Warm reminder: The above information is for health science reference only and cannot replace professional medical diagnosis. If you have related symptoms, please seek medical attention at a regular hospital promptly.