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Eur J Haematol. 2010 Jan 1;84(1):79-83. doi: 10.1111/j.1600-0609.2009.01307.x.

Primary cutaneous diffuse large B-cell lymphoma, leg type, with features simulating POEMS syndrome.

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Department of Internal Medicine, Osaka Medical College, Takatsuki City, Japan.


A 91-year-old woman presented with a rapidly proliferative cutaneous lesion on the left lower limb, which was identified as a primary cutaneous diffuse large B-cell lymphoma (PCLBCL), leg type, on biopsy. The patient also showed complications of hepatomegaly, endocrinopathy, edema, skin change, and polyneuropathy without monoclonal plasma cell proliferative disorder, and was therefore diagnosed with POEMS-like syndrome owing to the lack of monoclonal plasma cell proliferative disorder. Levels of serum vascular endothelial growth factor (VEGF) and interleukin-6 (IL-6) were high with the lymphoma cells immunostained positively for VEGF and IL-6. To the best of our knowledge, this is the first case report of PCLBCL, leg type, with POEMS-like syndrome. The findings in this case suggest that the symptoms of POEMS-like syndrome might be caused by the cytokines produced by the lymphoma cells. Furthermore, a wider range of diagnostic criteria associated with the result of abnormal secretion of cytokine may have to be considered for the diagnosis and evaluation of patients with possible POEMS syndrome, as against the present criteria specifying monoclonal plasma cell proliferative disorder as the essential criterion.

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