HIV-Associated NK/T-Cell Lymphomas: A Review of 93 Cases.

Introduction: NK/T-cell lymphomas (NKTCL) are being reported with increased frequency in HIV-infected patients in the HAART era. However, there has been no comprehensive review of these emerging HIV-associated lymphomas. We present, to our knowledge, the first comprehensive review of the epidemiology, clinical and pathologic features, therapeutic options, and outcome in HIV-associated NKTCL.

Methods: A total of 93 cases of NKTCL in HIV-infected individuals were identified in a literature review using PubMed and cited articles from 1996 until March 2007. Data regarding epidemiology (country), demographics (age, gender), clinical features (presentation, tumor location), HIV status (CD4, AIDS-defining illness, coinfections), lymphoma (WHO/EORTC subtype, immunophenotype, Ann Arbor stage), treatment, and outcome were extracted.

Results: Cases were reported worldwide (USA, Europe, South America, Asia), with a 15x increased incidence compared to the general population. Patients were of median age 38 years (range 1–63) at presentation, exhibited a 4:1 male predominance, and had a median CD4 count of 184 cells/mm³. Up to 54% had a prior AIDS-defining illness. The most common clinical findings were lymphadenopathy, B symptoms, erythroderma and pruritus. All subtypes were noted including Peripheral T-cell (n=36), Cutaneous T-cell including Mycosis fungoides (n=25), Anaplastic Large Cell (n=13), Adult T-cell Leukemia/Lymphoma (n=8), NK cell (n=4), and other lymphomas (2 AILD, 2 Enteropathy-associated, 1 PEL, 1 Intravascular). Most expressed CD45RO and CD3 antigens, CD4>CD8, CD30 in a subset (n=23), and EBV was detected in 20 cases (mainly extranodal PTCL, cutaneous ALCL, and NK cells). Most (74%) were extranodal (50% involving skin) and 26% nodal. Staging (60 patients) overall showed stage I (27%), II (3%), III (17%), and IV (53%).There was no standard therapeutic approach, with 46% given one modality, 17% multimodal treatment, and 12% were untreated in reported cases. HAART was rarely used. Death occurred in 55% of patients and median overall survival (83 patients) was 1.1 years from the time of diagnosis.

Conclusions: NKTCL afflicted HIV-infected persons are likely to be young males with AIDS who have a CD4 count under 200 cells/mm³ at presentation. They are also prone to present with extranodal disease, skin involvement, and at an advanced stage. Although a standard treatment approach is still required, the prognosis of HIV-associated NKTCL remains poor. The role of HAART needs to be further explored.