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Multicentric Castleman Disease: unusual clinical presentations and outcome in 6 recent cases

Multicentric Castleman Disease: unusual clinical presentations and outcome in 6 recent cases Ch. Martin, D. Konopnicki, S. De Wit, N. Clumeck Saint-Pierre University Hospital, Brussels, Belgium. E-mail : maladiesinfectieuses@stpierre-bru.be. 6 patients. Background

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Multicentric Castleman Disease: unusual clinical presentations and outcome in 6 recent cases

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  1. Multicentric Castleman Disease: unusual clinical presentations and outcome in 6 recent cases Ch. Martin, D. Konopnicki, S. De Wit, N. Clumeck Saint-Pierre University Hospital, Brussels, Belgium. E-mail : maladiesinfectieuses@stpierre-bru.be 6 patients • Background • MulticentricCastleman Disease (MCD) is a rare HIV-associated disease described mostly in caucasian homosexual men not treated for HIV. The precise incidence is unknown but some reports suggest a recent increasing possibly due to better diagnosis and awareness of clinicians. • Clinical presentation of MCD is polymorphic and sometimes fulminant. • It is a polyclonal lymphoproliferative disorder but monoclonal plasmablasticmicrolymphomas are often described in biopsied lymph nodes and risk for lymphomatousplasmablastic transformation is important (Oksenhendler2002). Methods We describe 6 HIV-positive patients with MCD +/- plasmablastic lymphoma transformation diagnosed and managed in our institution during the last 3 years (2008-2011) and compare our data’s with series of MCD described in the literature. 4 homosexuel men 2 africanheterosexuel women Results 3 MCD with lymphomatous transformation 1 MCD with lymphomatous transformation 1 pulmonary MCD • Symptoms: • Lymph nodes 2/6 • Anaemia with irregular antibody 3/6 • B symptoms 4/6 • Respiratory insufficiency 1/6 • Palatin mass 1/6 • Association with Kaposi Sarcoma 3/6 • (2 stomach, 3 lymph node capsule) • Transformationin plasmablastic lymphoma 4/6 • Bone marrow: No invasion 5/6 • B monoclonality 1/6 • MCD flare-up after HAART initiation 1/2 • Mortality 2/6 (33%) MCD 1 fulminant death Post-mortem diagn. 3 R-CHOP +/- valganciclovir 3 CR alive: FU 29-16-16 months 1 R-CHOP 1 CR alive: FU 18 months 1 R-CHOP then Etoposide PR Death at13 months. R-CHOP= Rituximab and CHOP. CR = Complete remission. PR= Partial remission Unusual findings: 2/6 heterosexual African women, 4/6 HIV-RNA<20 under HAART 1/2 flare-up of symptoms after HAART initiation, coexistent lymphoma 66%, 4/4 lymphomas treated with R-CHOP alive. Conclusion • We describe unusual presentation and outcome of Multicentric Castleman Disease : • Epidemiology: 33% in heterosexual African women and 66% in patients with undetectable HIV-RNA under HAART . • Presentation: plasmablastic lymphoma transformation was more frequent than reported in literature (66% vs 6.8% in Mylona 2008) . • Prognosis was better in patients with lymphoma treated by R-CHOP (4/4 alive in complete remission) than in patients without lymphoma (2/2 deaths). • We suggest to implement a Belgian protocol to collect MCD characteristics from the post-HAART era and to treat MCD following recent recommendations.

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