Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0235886 (leg edema)
 674 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome is a rare multi-systematic disorder of uncertain etiology, if associated with multicentric Castleman's disease, it can lead to a more serious condition. We here presented a case of polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome in a 37-year-old male patient who initially presented with progressive lower limb weakness accompanied by pain, low skin temperature, and hyperpigmentation. He was admitted with increasingly serious dyspnea and lower leg edema. Fluid of serous cavities in the patient were also indicated in ultrasonic inspection and X-ray. Furthermore, biopsy of a left axillary lymph node showed mixed hyaline-vascular and plasma cell type of multicentric Castleman's disease. Administration of bortezomib (Velcade) (1.3 mg/m(2) on days 1, 4, 8 and 11 of a 21-day cycle) combined with thalidomide (100 mg/day and 21-day cycle) dramatically improved the condition of this disease. Of note, in our study, combination therapy of bortezomib and thalidomide successfully improved the condition of the patient with polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome associated with multicentric Castleman's disease, suggesting that the combination therapy may be an effective therapeutic strategy for the intractable polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome associated with multicentric Castleman's disease.
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PMID:Successful treatment with bortezomib and thalidomide for POEMS syndrome associated with multicentric mixed-type Castleman's disease. 2189 Jun 54

A 48-year-old woman was hospitalized because of severe thrombocytopenia, leg edema, and fever. Intravenous immunoglobulin therapy was administered, but no efficacy was obtained. Her bone marrow was dry-tap, and fibrosis was found in the biopsy specimens. A positron emission tomographic study showed FDG-avid lymphadenopathy and hepatomegaly. Biopsy specimens of axillary lymph nodes showed Castleman's disease-like findings. Since she then developed severe proteinuria and massive pleural effusion, steroid therapy was started, providing temporary relief of symptoms other than the thrombocytopenia. However, rapid worsening of her general condition prompted us to attempt rituximab as salvage therapy. The pleural effusion, edema, and proteinuria disappeared soon after starting rituximab administration. Platelet counts also normalized and fibrosis of the bone marrow showed amelioration. Recently, a variant of multicentric Castleman's disease, termed the TAFRO syndrome, has been proposed, and our patient's features fit the diagnosis of this syndrome. Rituximab might be considered as a therapeutic option in such cases.
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PMID:[Efficacy of rituximab for TAFRO syndrome, a variant type of multicentric Castleman's disease]. 2468 40