POEMS Syndrome: A Report of 14 Cases and Review of the Literature

Abstract

POEMS syndrome is a rare paraneoplastic disorder associated with an underlying plasma cell dyscrasia presenting polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. This study reviewed the clinical characteristics of 14 POEMS patients in Zhongshan hospital. The ratio of male to female was 9 : 5, and the average age was 47.1 years. The clinical manifestations were various, including motorial symptoms (weakness), sensory symptoms (numbness), lymphadenopathy, edema, abdominal distention, and skin hyperpigmentation. Imaging studies and laboratory tests also exhibited hepatomegaly, splenomegaly, thrombocytosis, endocrinopathy, and positive serum immunofixation in most patients. In addition, increased plasma cells in bone marrow and Castleman Disease were found in bone marrow and lymph nodes biopsies. All the eight follow-up patients were treated with alkylator-based combination chemotherapy or corticosteroids and thalidomide, with or without autologous stem cell transplantation. Unfortunately, two patients died three or four years after diagnosis of POEMS syndrome. The others showed response to therapy to some extent, but not completely remission. Currently, treatments for POEMS include radiation to the plasmacytoma, and systemic therapy is indicated. Low-dose alkylators with or without corticosteroids are effective in some patients. However, high-dose chemotherapy with auto-SCT dramatically improved symptoms and outcomes for POEMS patients.

POEMS syndrome presenting with colonic pseudoobstruction and calciphylaxis

“…Based on case reports, highdose chemotherapy with autologous stem cell transplant seems effective in improving symptoms. 13 Our patient was treated with cyclophosphamide, bortezomib and dexamethasone followed by autologous stem cell transplant.…”

Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin abnormalities (POEMS) Syndrome in a Filipino Nurse Returning to Work Following Bortezomib Treatment: A Case Report

“…[7] This was also seen in patients subjected only to corticosteroids for treatment. [8] There is no standard first-line therapy for newlydiagnosed POEMS syndrome but current treatment approaches provide a good prognosis based only on one randomized controlled study that has been completed. Treatment for newly diagnosed POEMS syndrome depends on the extension of disease.…”

Long‐term durable efficacy of autologous stem cell transplantation in POEMS syndrome

“…1 Due to its rarity, the standard of care is not well established in the management of the disease. Recently, multiple studies have reported on the durable responses with autologous hematopoietic stem cell transplantation (auto-HCT), [2][3][4][5][6][7][8] which is accepted as standard treatment approach in eligible patients. 9 We had previously reported on the role of auto-HCT in a smaller cohort of POEMS patients.…”