Authors: Linus Angenendt Andrea Kerkhoff Stefanie Wiebe JanHenrik Mikesch Annika Rudat Fabian Wötzel Christian Wenning Christoph Schliemann Torsten Kessler Michael Schäfers Eva Wardelmann Matthias Stelljes Wolfgang E Berdel
Publish Date: 2015/03/21
Volume: 94, Issue: 7, Pages: 1241-1243
Abstract
Multicentric Castleman’s disease MCD is a rare lymphoproliferative disorder characterized by hypercytokinemia undulating signs of systemic inflammation generalized lymphadenopathy hepatosplenomegaly and in severe cases vascular leak syndrome VLS organ failure and death 1 It is categorized into HHV8positive MCD HMCD including HIV patients and idiopathic MCD iMCD Therapeutic monoclonal antibodies are the mainstay of treatment Whereas the antiCD20 antibody rituximab is preferentially advocated for HMCD 2 3 the IL6 disrupting antibodies tocilizumab and siltuximab are for iMCD 4 We report the first case to our knowledge of a patient with severe iMCD responding with repeated longlasting complete remissions CR to rituximab monotherapy and after failure a complete remission to allogeneic stem cell transplantation alloSCT
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