POEMS syndrome: A study of 25 cases and a review of the literature

Soubrier, Martin; Dubost, Jean‐Jacques; Sauvezie, B

doi:10.1016/0002-9343(94)90350-6

Cited by 314 publications

(301 citation statements)

References 65 publications

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“…The course of POEMS syndrome is usually chronic with modern estimated median survivals of nearly 14 years [48,58] Only fingernail clubbing, extravascular volume overload-i.e., effusions, edema, and ascites [48], respiratory symptoms [58], pulmonary hypertension [80] impaired DLCO, and papilledema [52] have been associated with a significantly shorter overall survival. The number of POEMS features does not affect survival [5,10]. Patients who are candidates for radiation therapy have a better overall survival (Fig.…”

Section: Risk Stratificationmentioning

confidence: 98%

“…The variability between series is most likely a function retrospective reporting-i.e. if a physician does not order a test or chart a finding, it will not be captured-and local practices, and promptness of diagnosis, rather than ethnic differences [2,3,5,7,48]. The neuropathy is the dominant characteristic.…”

Section: Diagnosismentioning

confidence: 99%

“…A whole skin examination should be performed looking for hyperpigmentation, a recent out-cropping of hemangioma, hypertrichosis, dependent rubor and acrocyanosis, white nails, sclerodermoid changes, facial atrophy, flushing, or clubbing [2,[4][5][6][7][8]54,55] Rarely calciphylaxis is also seen [56,57]. Respiratory complaints are usually limited given patients' neurologic status impairing their ability to induce cardiovascular challenges.…”

Section: Diagnosismentioning

confidence: 99%

“…The symptoms can range from simple B-symptoms to various autoimmune phenomenon to a frank POEMS syndrome (Fig. 1) [3,5,48,. Several published cases of "interesting features" associated with Castleman disease are likely cases of POEMS syndrome [130][131][132][133].…”

Section: Relationship To Castleman Disease and Castleman Disease Varimentioning

confidence: 99%

“…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%

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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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