Rituximab and Castleman disease and Kaposi's sarcoma

Background

Castleman disease was initially described as benign localized lymph nodes found primarily in the mediastinum of asymptomatic patients; additional types were recognized that extend the spectrum of this heterogeneous group of diseases. Optimal standard therapies have not been established. Rituximab is an anti-CD20 monoclonal antibody that has demonstrated efficacy in patients with various lymphoid malignancies. It is an unusual complication in patients with HIV-1 and HHV-8 infection, but it should be included in the differential diagnosis of patients who exhibit a relapsing systemic inflammatory syndrome and lymphoadenopathy.

Methods

A 47 year-old patient, HIV seroconversion known since 1997, evaluated for the first time in 1997 (CD4 450) and in 1999 (CD4 278). Up to 2006 follow-up was lost, but receiving during two years antiretroviral treatment (unknown). Re-evaluated again in October 2006 (CD4 75, viral load 290,000 copies/ml), and began treatment with abacavir (ABC) + 3TC + efavirenz. In 15 days time he presented systemic complaints of fever, weight loss and night sweats. ABC was adjourned suspecting hypersensitivity reaction. Also, he was diagnosed with splenic Non-Hodgkin's lymphoma after splenectomy. Treatment was re-initiated with 3TC + ddI + EFV. CT revealed pathologic adenopathies in abdomen and armpits. During the following months similar symptoms recurred. A biopsy on one of the cutaneous injuries led to findings characteristic of Kaposi's sarcoma, and biopsy on armpit and abdominal lymph nodes of Castleman disease and infiltration by Kaposi's sarcoma, and confirmed the presence of HHV-8. After these diagnosis treatment with rituximab began (375 mg/m2, 4 weekly doses).

Summary of results

After treatment the patient is asymptomatic (follow-up 7 months). Control CT, one month before finishing treatment (CD4 140, undetectable CV), revealed multiple subcentimetric adenopathies in the abdominal tracking.

Conclusion

Rituximab has been an effective therapy in our patient, without other chemotherapy treatments.