Plasma Cell Dyscrasia with Polyneuropathy, Organomegaly, Endocrinopathy, M Protein, and Skin Changes

Bardwick, P A; Zvaifler, Nathan J.; Gill, Gordon N.; Newman, Denis; Greenway, Guerdon; Resnick, Donald

doi:10.1097/00005792-198007000-00006

Cited by 634 publications

(290 citation statements)

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“…The endocrinological disorders observed in a large series of POEMS syndrome cases were amenorrhea, gynecomastia, impotence, glucose intolerance, hyperestrogenemia, hypothyroidism and hyperprolactinemia [2][3][4][5], but few reports have described a thorough evaluation of the endocrine system, and the mechanisms involved in the abnormalities…”

Section: Discussionmentioning

confidence: 99%

Improvement in Gonadotropin Secretion after Corticosteroid Therapy in a Case of POEMS Syndrome.

1998

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Abstract.We report a case of POEMS syndrome with various endocrine dysfunctions.A 49-year-old man was admitted to our hospital for pretibial edema and general fatigue. he showed an improvement in gonadotropin secretion, but no considerable change in the secretion of the other hormones.To our knowledge, this is the first case that showed an improvement in gonadotropin secretion as a result of corticosteroid therapy in POEMS syndrome.

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Section: Discussionmentioning

confidence: 99%

Improvement in Gonadotropin Secretion after Corticosteroid Therapy in a Case of POEMS Syndrome.

1998

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“…Between 11 and 30% of POEMS patients who have a documented clonal plasma cell disorder also have documented Castleman disease or Castleman-like histology [1,3,5,7,48]. In 30 patients with POEMS syndrome, 19 of 32 biopsied lymph nodes showed angiofollicular hyperplasia typical of Castleman disease [3].…”

Section: Relationship To Castleman Disease and Castleman Disease Varimentioning

confidence: 99%

POEMS syndrome: Update on diagnosis, risk‐stratification, and management

2015

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Disease overview: POEMS syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. Diagnoses are often delayed because the syndrome is rare and can be mistaken for other neurologic disorders, most commonly chronic inflammatory demyelinating polyradiculoneuropathy. POEMS syndrome should be distinguished from the Castleman disease variant of POEMS syndrome, which has no clonal PCD and typically little to no peripheral neuropathy but has several of the minor diagnostic criteria for POEMS syndrome.Diagnosis: The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma cell disorder, and at least one of the minor criteria.Risk stratification: Because the pathogenesis of the syndrome is not well understood, risk stratification is limited to clinical phenotype rather than specific molecular markers. The number of clinical criteria is not prognostic, but the extent of the plasma cell disorder is. Those patients with an iliac crest bone marrow biopsy that does not reveal a plasma cell clone are candidates for local radiation therapy; those with a more extensive or disseminated clone will be candidates for systemic therapy.Risk‐adapted therapy: For those patients with a dominant sclerotic plasmacytoma, first line therapy is irradiation. Patients with diffuse sclerotic lesions or disseminated bone marrow involvement and for those who have progression of their disease 3–6 months after completing radiation therapy should receive systemic therapy. Corticosteroids are temporizing, but alkylators are the mainstay of treatment, either in the form of low dose conventional therapy or high dose with stem cell transplantation. Lenalidomide shows promise with manageable toxicity. Thalidomide and bortezomib also have activity, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy. The benefit of anti‐VEGF antibodies is conflicting. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes. Am. J. Hematol. 90:951–962, 2015. © 2015 Wiley Periodicals, Inc.

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“…The POEMS syndrome is a rare disorder originally related to polyneuropathy (P), organomegaly (O), endocrinopathy (E), serum monoclonal protein (M), and skin changes (S) [1,2]. Some patients may have osteosclerotic bone lesions, papilledema, pulmonary hypertension, and renal involvement (mesangiocapillary-like glomerulonephritis) [3,4].…”

Section: Introductionmentioning

confidence: 99%

Thalidomide in POEMS syndrome: Case report

et al. 2004

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We report a patient with incapacitating POEMS syndrome characterized by serum monoclonal protein, polyneuropathy, organomegaly, endocrinopathy, mesangiocapillary glomerulonephritis, massive ascites formation, and pulmonary hypertension. A dramatic improvement in the clinical condition occurred after administration of thalidomide, a drug with known anti-angiogenetic, anti-proliferative, and anti-cytokine properties.

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Plasma Cell Dyscrasia with Polyneuropathy, Organomegaly, Endocrinopathy, M Protein, and Skin Changes

Cited by 634 publications

References 0 publications

Improvement in Gonadotropin Secretion after Corticosteroid Therapy in a Case of POEMS Syndrome.

Improvement in Gonadotropin Secretion after Corticosteroid Therapy in a Case of POEMS Syndrome.

POEMS syndrome: Update on diagnosis, risk‐stratification, and management

Thalidomide in POEMS syndrome: Case report

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