Zhonghua nei ke za zhi · 2006
[A clinical analysis of 32 cases of POEMS syndrome].
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Zhu Wei-guo, Wang Yu, Zeng Xue-jun, Wang Qiang, Shen Ti
Аннотация
OBJECTIVE: To compare the clinical features of POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes) syndrome patients from Peking Union Medical College Hospital (PUMCH) with those of patients in previous reports.
METHODS: Thirty-two patients with POEMS syndrome were identified in PUMCH over the last two decades. The clinical features of these patients were compared with those in other large series from Japan, China, and the United States.
RESULTS: The average age at presentation was 45.8 years; 22 were men. All 32 patients had polyneuropathy and decreased knee-jerk reflex. Organomegaly was present in 31 patients. Nine patients underwent lymph node biopsy and 5 of them were proven to have Castleman's disease. Twenty-six patients had at least one endocrine abnormality at time of presentation, not including more commonly prevalent diseases like diabetes mellitus or hypothyroidism. Seventy-two percent (18/25) of the patients had a detectable monoclonal protein with serum immunofixation, but only 16.7% (4/24) had positive results with serum protein electrophoresis. Skin changes were documented in 30 patients. In comparison with other series, we found a higher prevalence of organomegaly and papilloedema. The rates of M protein level, however, were similar.
CONCLUSIONS: Most features of our series are similar to those from previous foreign series. The prevalence of M protein in our study was similar to that in previous foreign series, but was not as low as reported in previous Chinese studies. Immunofixation and immunohistochemical staining of bone marrow biopsies are probably more sensitive in detecting M protein as compared with serum protein electrophoresis.
METHODS: Thirty-two patients with POEMS syndrome were identified in PUMCH over the last two decades. The clinical features of these patients were compared with those in other large series from Japan, China, and the United States.
RESULTS: The average age at presentation was 45.8 years; 22 were men. All 32 patients had polyneuropathy and decreased knee-jerk reflex. Organomegaly was present in 31 patients. Nine patients underwent lymph node biopsy and 5 of them were proven to have Castleman's disease. Twenty-six patients had at least one endocrine abnormality at time of presentation, not including more commonly prevalent diseases like diabetes mellitus or hypothyroidism. Seventy-two percent (18/25) of the patients had a detectable monoclonal protein with serum immunofixation, but only 16.7% (4/24) had positive results with serum protein electrophoresis. Skin changes were documented in 30 patients. In comparison with other series, we found a higher prevalence of organomegaly and papilloedema. The rates of M protein level, however, were similar.
CONCLUSIONS: Most features of our series are similar to those from previous foreign series. The prevalence of M protein in our study was similar to that in previous foreign series, but was not as low as reported in previous Chinese studies. Immunofixation and immunohistochemical staining of bone marrow biopsies are probably more sensitive in detecting M protein as compared with serum protein electrophoresis.