POEMS syndrome: 2021 Update on diagnosis, risk‐stratification, and management

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. 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. Risk factors include low serum albumin, age, pleural effusion, pulmonary hypertension, and reduced eGFR. Risk‐adapted therapy For those patients with a dominant 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. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes.

POEMS Syndrome: Real World Experience in Diagnosis and Systemic Therapy - 108 Patients Multicenter Analysis

“…POEMS (Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) syndrome is a rare plasma cell disorder [1]. It is actually a paraneoplastic syndrome caused by an underlying clonal plasma cell disorder which is difficult to recognize, underdiagnosed, and due to the rarity of the disease, outcome analyses are challenging and usually require multicenter cooperation.…”

“…The association of the pro-inflammatory cytokine, vascular endothelial growth factor (VEGF) in POEMS is well documented [1,2]. Although VEGF levels decrease with therapeutic intervention, there may not be a direct correlation with clinical response.…”

“…Treatment approaches are based upon the extent of plasma cell infiltration and end-organ damage utilizing serological parameters, bone marrow evaluation and radiographic findings. In localized disease, radiotherapy is recommended [1,2]. However, this is not considered curative for patients with low burden bone marrow infiltration indicative of systemic disease.…”

“…However, this is not considered curative for patients with low burden bone marrow infiltration indicative of systemic disease. The therapeutic objective is to eliminate the underlying -the plasma cell disorder since the depth of hematological response determines PFS [1,2]. Of note, as in amyloidosis, organ response may be extensively delayed compared to hematological responses [1].…”

“…The therapeutic objective is to eliminate the underlying -the plasma cell disorder since the depth of hematological response determines PFS [1,2]. Of note, as in amyloidosis, organ response may be extensively delayed compared to hematological responses [1].…”