Journal of Neurology and Neuroscience

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Primary iffuse Large B-Cell Lymphoma with Features Simulating POEMS Syndrome: Case Report and Review Of The Literature

Ahmed Mili, Salma Naija, Asma Nasr, Manel Ben Halima, Anis Hassine, Sana Ben Amor, Nesrine Ben Sayed, Yosra Ben Youssef, Abderrahim Khélif, Dorra Chiba, Badreddine Sriha, Moncef Mokni

POEMS syndrome is a rare systemic affection of paraneoplastic origins. The main manifestations include polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. This disease is commonly associated with plasma cell dyscrasia or Castleman disease. A 51-year-old-man presented with a four-month history of gait impairment, four-limb sensorimotor deficit, general areflexia and facial diplegia. The diagnosis of chronic polyradiculoneuritis was made based on Cerebrospinal fluid (CSF) analysis and electromyography study (EMG). At the time of initial evaluation, physical examination displayed enlarged lymph nodes, hepatosplenomegaly, endocrinopathy and skin changes. Complete Blood Count did not reveal significant monoclonal plasma cell proliferative disorder. The biopsy of the adenopathy identified a large B-cell lymphoma. We concluded a POEMS-like syndrome due to the lack of monoclonal plasma cell proliferative disorder. To our knowledge, this is the third reported case of B-cell Lymphoma associated with POEMS features. Our findings may suggest that POEMS syndrome has an unusual presentation in patients with diffuse large B-cell lymphoma even in the absence of monoclonal plasma cell proliferative disorder considered as the essential criterion.