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

الملخص EN

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.