MULTICENTRIC CASTLEMANS-DISEASE IN HIV-INFECTION - A CLINICAL AND PATHOLOGICAL-STUDY OF 20 PATIENTS

Objectives: To describe, in a retrospective study, the clinical and pa thological spectrum of multicentric Castleman's disease (MCD) in HIV i nfection. Patients: The diagnosis of CD was established by lymph node biopsy in 20 HIV-infected patients. All patients had been HIV-infected by sexual contact. At diagnosis, HIV infection was asymptomatic in ei ght patients and Kaposi's sarcoma was present in 12. Mean +/-SD CD4+ c ell count was 156 +/- 99 x 10(6)/l. Results: Patients were referred wi th a syndrome of fever and splenomegaly (100%), peripheral lymphadenop athy (90%), hepatomegaly (70%), severe weight loss (70%), respiratory symptoms (65%) and oedema (55%). Anaemia was a constant finding and se ven (35%) patients presented with pancytopenia. Serum markers of infla mmation were present in most patients: a high level of C reactive prot ein (90%), polyclonal hypergammaglobulinaemia (89%) and hypoalbuminaem ia (56%). The histological pattern of the lymph nodes was characterize d by small hyalinized germinal centres surrounded by concentric layers of small lymphocytes, vascular hyperplasia, hyalinized vessels and la rge interfollicular sheets of plasma cells. Five patients were classif ied as plasma cell type MCD and 15 as hyaline vascular/plasma cell (mi xed) type. Immunophenotyping studies (n = 13) demonstrated a polyclona l B-cell process. No linkage with Epstein-Barr virus (EBV) could be de monstrated immunohistochemically using an anti-latent membrane protein -1 monoclonal antibody (n = 16) or by RNA in situ hybridization with a n EBV-encoded RNA transcript-specific probe (n = 13). Remission was ob tained with low-dose and usually single agent chemotherapy in 16 patie nts. During follow-up, non-Hodgkin's lymphoma developed in two patient s and Kaposi's sarcoma in three. Fatal outcome occurred in 14 patients with a median survival of 14 months. Conclusion: MCD associated with HIV infection is a distinct clinico-pathological entity that can be di fferentiated from other types of HIV-associated systemic lymphoprolife rative disorders. It is very similar to MCD observed in non-HIV-infect ed patients, except for the high prevalence of pulmonary symptoms and for the stronger association with Kaposi's sarcoma. Single-agent chemo therapy with vinblastine is effective and may prolong survival.