Abstract
Multicentric Castleman disease (MCD) is a rare lymphoproliferative disorder that is increasingly diagnosed in patients with HIV infection. The course of the disease is unpredictable, ranging from a rapidly progressive form that can be fatal within weeks to a long course of a remitting and relapsing disease. Its principal manifestations are diverse but almost always include fever, malaise, lymphadenopathy and hepatosplenomegaly. Pulmonary manifestations are rare.
We report a case of HIV-associated MCD that relapsed after being on remission for 10 years. The patient presented with recurrent “attacks” of classical symptoms (fever, night sweats, polyadenopathy), accompanied by rhinorrhea, shortness of breath and cough. A diffuse interstitial pneumonitis was found on CT-scan of the chest, described as a ground-glass infiltrate with peri-lymphatic micronodules and interlobular thickening that affected mostly the inferior third of both lungs. A slight bilateral pleural effusion was also noted. Lung function tests were normal. Final diagnosis of MCD was made on lymph node biopsy. We first treated this patient with Rituximab alone which led to a rapid relief of his symptoms and radiologic improvement. However, as he relapsed twice following this treatment, each time with systemic symptoms accompanied by a radiologic progression of the diffuse interstitial pneumopathy, we opted for a combined regimen of Rituximab and chemotherapy (CHOP). He is still on remission (6 months) and a follow-up CT-scan shows an almost complete regression of the pulmonary interstitial anomalies. The long term management of MCD presenting with pulmonary involvement has yet to be defined, as long term prognosis.
- © 2012 ERS