POEMS (polyneuropathy, organomegaly, endocrinopathy, M‐protein and skin changes) syndrome in China

Cui, Rong; Huang, Xu; Shi, Quan; Tian, Chenglin; Liu, J. X.; Pu, Chuan Qiang

doi:10.1111/j.1445-5994.2010.02172.x

Cited by 19 publications

(14 citation statements)

References 14 publications

(44 reference statements)

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“…Among our patients, the splenomegaly was the most common symptom (84.6%), which mirrors to the report of Cui et al [10]. Nevertheless, Li et al [18] reviewed the clinical characteristics of 99 Chinese POEMS patients and found 74 patients with lymphadenopathy, 70 with splenomegaly, and 47 with hepatomegaly.…”

Section: Discussionsupporting

confidence: 86%

“…It is a multisystem disorder associated with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes [10, 11]. Elevation of proangiogenic and pro-inflammatory cytokines are the hallmark in the pathogenesis of this disorder [12–15].…”

Section: Discussionmentioning

confidence: 99%

“…The M-protein is usually IgG or IgA and almost of the lambda type [1, 10]. According to Dispenzieri et al [9], all patients had evidence of a monoclonal plasmaproliferative disorder, and 87% had a detectable M-protein in their serum or urine by immunofixation.…”

Section: Discussionmentioning

confidence: 99%

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

Zhang

Weng

et al. 2012

ISRN Gastroenterology

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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.

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Section: Discussionsupporting

confidence: 86%

Section: Discussionmentioning

confidence: 99%

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

Zhang

Weng

et al. 2012

ISRN Gastroenterology

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“…Bone lesions are hallmarks of POEMS syndrome and occurred in 95% of cases in a cohort in the United States (11) and 27%-46% in Chinese cohorts (11,(13)(14)(15). In our study, 74.7% of the patients had bone lesions, which contributed to detection of a greater number of tiny skeletal abnormalities on CT, compared with the traditional radiographic skeletal survey-43.3% of all the patients had 18 F-FDG-avid bone lesions.…”

Section: Discussionmentioning

confidence: 99%

“…Another feature that aids the differential diagnosis is multiple cavity effusion without metabolism distribution, especially when the percutaneous area is involved simultaneously, which is a rare manifestation in other systemic diseases. The ascitic fluid may be exudative, but the detailed mechanism is still unclear (13).…”

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confidence: 99%