POEMS syndrome

Update on diagnosis, risk-stratification, and management

Research output: Contribution to journalArticle

69 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)951-962
Number of pages12
JournalAmerican Journal of Hematology
Volume90
Issue number10
DOIs
StatePublished - Oct 1 2015

Fingerprint

POEMS Syndrome
Risk Management
Plasma Cells
Polyradiculoneuropathy
Giant Lymph Node Hyperplasia
Peripheral Nervous System Diseases
Vascular Endothelial Growth Factor A
Therapeutics
Plasma Cell Neoplasms
Radiotherapy
Clone Cells
Bone Marrow
Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Paraneoplastic Syndromes
Thrombocytosis
Papilledema
Thalidomide
Plasmacytoma
Alkylating Agents
Delayed Diagnosis

ASJC Scopus subject areas

  • Hematology

Cite this

POEMS syndrome : Update on diagnosis, risk-stratification, and management. / Dispenzieri, Angela.

In: American Journal of Hematology, Vol. 90, No. 10, 01.10.2015, p. 951-962.

Research output: Contribution to journalArticle

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