How I treat POEMS syndrome.
نویسنده
چکیده
POEMS syndrome is a paraneoplastic syndrome whose acronym stands for less than half of the defining features of the disease, that is, polyradiculoneuropathy, organomegaly, potentially including coexisting Castleman disease, endocrinopathy, monoclonal plasma cell neoplasm, and skin changes. The other important features include papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis, elevated VEGF, and abnormal pulmonary function. The diagnosis is based on having both the polyradiculoneuropathy and the monoclonal plasma cell disorder, and at least 1 of the other 3 major criteria (Castleman disease, sclerotic bone lesions, or elevated VEGF) and at least one minor criterion. The diagnosis is often delayed with intervening incorrect diagnoses of chronic inflammatory demyelinating polyradiculoneuropathy, myeloproliferative disorder, and monoclonal gammopathy of undetermined significance. Prompt treatment directed at the underlying plasma cell clone produces dramatic responses in the majority of patients. Although there are no randomized clinical trial data to direct best therapy, for patients with disseminated disease, high-dose chemotherapy with peripheral blood transplantation has yielded durable benefit, whereas radiation therapy is typically effective for patients with a more localized presentation. More universal recognition of and more scientific inquiry into the underpinnings of the disease will provide direction toward the best treatment strategies in the future.
منابع مشابه
How I treat How I treat POEMS syndrome
POEMS syndrome is a paraneoplastic syndrome whose acronym stands for less than half of the defining features of the disease, that is, polyradiculoneuropathy, organomegaly, potentially including coexisting Castleman disease, endocrinopathy, monoclonal plasma cell neoplasm, and skin changes. The other important features include papilledema, extravascular volume overload, sclerotic bone lesions, t...
متن کاملPoems Syndrome in A-10-year-old Gi̇rl: a Case Report Poems Sendromu: 10 Yaşinda Kiz Olgu
POEMS syndrome has been defined as an association of plasma celi dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes. In this report, we describe a case of POEMS syndrome. A 10-year-old girl presented with polyneoropathy, diabetes mellitus type I, double monoclonal gammopathy (IgA and IgG), hepatomegaly, cardiomegaly, hyperpigmentation, and hypertricosis. To ...
متن کاملگزارش یک مورد نشانگان پوئمز (POEMS SYNDROME) از ایران و مروری بر مقالاتی که در این زمینه منتشر شده است.
The crow-fukas, Takatsuki or POEMS syndrome (The acronym of Polyneuropathy, Organomegaly, endocrinopathy, M component and skin changes), a rare, multisystem disorder associated with osteosclerotic myeloma, is characterize by the combination of plasma cell discrasia with polyneuropathy, organomegaly, endocrinopathy, monoclonal(M) protein, skin changes, as well as various other sign, such...
متن کاملMarkedly elevated serum total N-terminal propeptide of type I collagen is a novel marker for the diagnosis and follow up of patients with POEMS syndrome.
متن کامل
POEMS syndrome, calciphylaxis and focal segmental glomerulosclerosis – VEGF as a possible link
BACKGROUND Polyneuropathy organomegaly endocrinopathy M-protein skin changes (POEMS) syndrome is a rare cause of polyneuropathy. Calciphylaxis, a severe disease leading to necrotic ulcers of the skin, is associated with POEMS syndrome and also with renal disease. This case report describes a patient with POEMS syndrome plus primary focal segmental glomerulosclerosis. CASE PRESENTATION A 27-ye...
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عنوان ژورنال:
- Blood
دوره 119 24 شماره
صفحات -
تاریخ انتشار 2012