POEMS syndrome is a rare paraneoplastic disorder Paraneoplastic Syndromes Paraneoplastic syndromes are symptoms that occur at sites distant from a tumor or its metastasis. Although the pathogenesis remains unclear, these symptoms may be secondary to substances secreted... read more and is probably caused by circulating immunoglobulins caused by a plasma cell disorder Overview of Plasma Cell Disorders Plasma cell disorders are a diverse group of disorders of unknown etiology characterized by Disproportionate proliferation of a single clone of B cells Presence of a structurally and electrophoretically... read more . Circulating cytokines (interleukin 1-beta, interleukin 6), vascular endothelial growth factor (VEGF), and tumor necrosis factor-alpha are also increased.
Symptoms and Signs of POEMS Syndrome
Patients may have the following:
Osteoclastic bone lesions
Polyneuropathy (peripheral motor and sensory manifestations)
Skin abnormalities (eg, hyperpigmentation, dermal thickening, hirsutism, angiomas, hypertrichosis)
Other symptoms and signs of POEMS syndrome may include edema, ascites, pleural effusion, papilledema, and fever. About 15% of patients with POEMS syndrome have associated Castleman disease (a lymphoproliferative disorder, some forms of which are associated with infection by HIV or human herpesvirus 8).
Diagnosis of POEMS Syndrome
Like other syndromes of undefined pathophysiology, POEMS syndrome is diagnosed based on the constellation of symptoms, signs and laboratory findings.
Patients must have both mandatory criteria and ≥ 1 major and ≥ 1 minor criteria.
Mandatory criteria (both):
Evidence of monoclonal plasma cell proliferation
Major criteria (≥ 1):
Elevated level of vascular endothelial growth factor (VEGF)
Sclerotic bone lesions
Minor criteria (≥ 1):
Endocrinopathy (other than diabetes and hypothyroidism)
Extravascular volume overload (eg, ascites, peripheral edema, pleural effusion)
Thrombocytosis or polycythemia
Treatment of POEMS Syndrome
Chemotherapy with or without hematopoietic stem cell transplantation
Treatment of POEMS syndrome consists of radiation therapy or chemotherapy for bony lesions or bone marrow involvement sometimes followed by autologous hematopoietic stem cell transplantation Hematopoietic Stem Cell Transplantation Hematopoietic stem cell (HSC) transplantation is a rapidly evolving technique that offers a potential cure for hematologic cancers ( leukemias, lymphomas, myeloma) and other hematologic disorders... read more (1 Treatment reference POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes) is a nonautoimmune polyglandular deficiency syndrome. POEMS syndrome is a rare paraneoplastic disorder... read more ). Five-year survival is about 60%.
1. Khouri J, Nakashima M, Wong S: Update on the diagnosis and treatment of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) syndrome. JAMA Oncol 7(9):1383-1391, 2021. doi: 10.1001/jamaoncol.2021.0586