, Volume 89, Issue 2, pp 217-219
Date: 28 Jul 2009

Efficacy of bortezomib in refractory form of multicentric Castleman disease associated to poems syndrome (MCD-POEMS variant)

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Dear Editor,

Castleman’s disease is a rare lymphoproliferative disorder with a variable clinical course [1]. Multicentric Castleman disease (MCD) presents with generalized lymphadenopathy, hepatosplenomegaly, fever, and night sweats, and aggressive treatment is required [2]. Viral infections, autoimmunity disorders, and altered cytokine (IL-6) regulation have been implicated in CD pathogenesis [2, 3]. The association between CD and POEMS syndrome is well known [4].

A 49-year-old male, diagnosed of depressive disorder, and dysmyelinating peripheral polyneuropathy presented in June 2001 with asthenia, anorexia, generalized edema, and diarrhea. Physical examination revealed rash, left axial adenopathy, and distal dysesthesia in superior extremities. Normochromic anemia with slight leukocytosis was observed. Total protein and immunoglobulin levels were normal. No monoclonal protein was detected. Beta-2 microglobulin was increased to 3.19 mg/L (normal = 0.3–2.1 mg/L). LDH levels and hepatic a