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

Abstract

Disease OverviewPOEMS syndrome is a life‐threatening condition due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder, 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.DiagnosisThe 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 StratificationBecause the pathogenesis of the syndrome is not well understood, risk stratification is limited to clinical phenotype rather than specific molecular markers. Risk factors include low serum albumin, age, pleural effusion, pulmonary hypertension, and reduced estimated glomerular filtration rate.Risk‐Adapted TherapyFor those patients with a dominant plasmacytoma, first‐line therapy is irradiation. Patients with diffuse sclerotic lesions or disseminated bone marrow involvement should receive systemic therapy. Corticosteroids are temporizing, but alkylators and lenalidomide are the mainstays of treatment, the former either in the form of low‐dose conventional therapy or as high‐dose conditioning for stem cell transplantation. Thalidomide and bortezomib also have activity, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy. Daratumumab combinations also appear promising based on case series. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes.

Electrodiagnosis of polyneuropathy, organmegaly, endocrinopathy, M-protein, skin changes syndrome patients with peripheral neuropathy and potential-related risk factors

“…All patients fulfilled the following criteria for the diagnosis of POEMS syndrome: (1) mandatory major criteria including polyneuropathy and monoclonal plasma cell disorder; (2) other major criteria including the presence of elevated VEGF, sclerotic bone lesions, or Castleman syndrome; and (3) minor criteria including organomegaly, extravascular volume overload, endocrinopathy, skin changes, papilledema, and thrombocytosis. These criteria were used to establish the diagnosis of POEMS syndrome in our patient cohort 4 . We collected 46 patients who completed EMG due to the appearance of peripheral nerve symptoms, but according to the exclusion criteria of individuals with other possible underlying response of PN, including diabetes mellitus, advanced renal disease, alcohol, vitamin B12 deficiency, and adverse reactions to medicine were excluded.…”

“…Owing to its rarity, POEMS syndrome is frequently misdiagnosed as other neurological conditions, with chronic inflammatory demyelinating polyradiculoneuropathy being a common misdiagnosis. 4 The diagnosis of POEMS syndrome heavily relies on the presence of peripheral neuropathy (PN), and electromyography (EMG) proves to be a valuable tool in this regard. However, the potential use of EMG data to evaluate the extent of PN in individuals with POEMS syndrome has not been thoroughly investigated.…”

An open‐label, prospective trial to evaluate the efficacy and safety of ixazomib in combination with cyclophosphamide and dexamethasone in patients with newly diagnosed POEMS syndrome

“…An open-label, prospective trial to evaluate the efficacy and safety of ixazomib in combination with cyclophosphamide and dexamethasone in patients with newly diagnosed POEMS syndrome diagnosis of POEMS was made according to Dispenzieri criteria. 1 The inclusion and exclusion criteria were showed in the supplementary data. This study was registered with the Chinese Clinical Trials Registry (ChiCTR2000030072).…”

“…2024;205:478-482. wileyonlinelibrary.com/journal/bjh I N TRODUC TION POEMS syndrome, characterized as polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes, is a rare type of plasma cell disorder. 1 So far, there is no standard treatment for POEMS syndrome. Previous research has highlighted the therapeutic potential of different classes of drugs, including immunomodulatory drugs, proteasome inhibitors and alkylating agents.…”