title: Human immunodeficiency virus-associated multicentric Castleman disease refractory to antiretroviral therapy: clinical features, treatment and outcome.
abstract: Human immunodeficiency virus (HIV)-associated multicentric Castleman disease (MCD) is a lymphoproliferation associated with human herpes virus-8 (HHV-8). Optimal treatment in patients not responding to antiretroviral therapy (ART) is undefined. We report 12 patients with ART refractory HIV-MCD. Patients with HIV-MCD were identified and baseline characteristics, treatment and outcome considered. Median CD4 count at HIV-MCD diagnosis was 295 (60-950) cells/mL. All patients had waxing and waning systemic symptoms, lymphadenopathy and/or splenomegaly, with non-Hodgkin lymphoma (NHL) in three. Treatment included: anti-HHV-8 therapy, n = 8; alone, n = 4; with systemic chemotherapy (CT) ± immunotherapy (IT), n = 4; CT ± IT only, n = 2. Initial median HHV-8 viral load (VL) was 7 × 10(4) copies/mL and at follow-up < 40 in 6/7 survivors; and 403-7.2 × 10(6) in 4/5 who died. One patient developed NHL despite an HHV-8 VL < 40. HIV-MCD is challenging to treat. Suppression of plasma HHV-8 VL did not prevent development of NHL. Anti-HHV-8 therapy should probably be considered adjunctive to cytotoxic therapies.
authors.affiliation: Hematology Program, University of British Columbia and King Saud University , Riyadh , Saudi Arabia.
authors.forename: Musa
authors.initials: M
authors.lastname: Alzahrani
authors.forename: Mark C
authors.initials: MC
authors.lastname: Hull
authors.forename: Christopher
authors.initials: C
authors.lastname: Sherlock
authors.forename: Deborah
authors.initials: D
authors.lastname: Griswold
authors.forename: Chantal S
authors.initials: CS
authors.lastname: Leger
authors.forename: Heather A
authors.initials: HA
authors.lastname: Leitch
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D023241:Antiretroviral Therapy, Highly Active; D001706:Biopsy; D018791:CD4 Lymphocyte Count; D005871:Castleman Disease / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D019468:Disease Management; D005260:Female; D015658:HIV Infections / Q000150:complications* / Q000188:drug therapy / Q000821:virology; D006566:Herpesviridae Infections / Q000150:complications; D019288:Herpesvirus 8, Human; D006801:Humans; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome; D019562:Viral Load
keywords: ART; HIV; Multicentric Castleman disease