Mark Levis, M.D., Ph.D. and Nina Wagner, M.D.
- Little specific information regarding incidence of HIV-related lymphoma in sub-Saharan Africa.
- From available studies, there appears to be low rate or no association at all with Hodgkin's lymphoma.
- In comparison, there is strong association between HIV and increased risk for non-Hodgkin's lymphoma.
- Burkitt's lymphoma due to EBV remains a common childhood tumor in Africa regardless of HIV status.
Zambia Information Author: Paul Auwaerter, M.D.
- Almost always EBV-associated, unlike in HIV-negative pts.
- Risk of developing HL is 8-10 x higher in HIV-infected persons compared with general population. Incidence has unexpectedly increased in HAART era.
- Associated with higher CD4 counts (~300) vs. NHL.
- Pts often have diffuse lymphadenopathy but with uninvolved nodes. Lymphadenopathy can make Dx and accurate staging difficult.
- Tends to present with advanced stage and in unusual locations, especially bone marrow.
- Frequently presents with "B Sx," e.g., night sweats & weight loss, which are commonly associated with other AIDS-related illnesses.
- Histologic subtypes in HIV+ population are less favorable mixed cellularity and lymphocyte-depleted variants.
- Tumor cells show strong expression of EBV latent membrane protein-1 (LMP-1).
- Bx of node or affected organ essential. Fine needle aspiration (FNA) is NOT desired mode of Dx, as usually non-diagnostic in Hodgkin's.
- Immunophenotyping of paraffin block or flow cytometric analysis of cell surface markers (CD3, CD5, CD10, CD15, CD20, CD30, Ki-67) in combination with molecular studies (EBV or HHV-8, cytogenetics, FISH).
- Staging workup should include chest/abdomen/pelvic CT, bone marrow Bx and aspirate, LP, CBC, LDH and complete chemistries.
- Role of PET imaging in HIV-associated lymphomas is still unclear, though frequently obtained.
- International Prognostic Scores (IPS) useful in predicting outcome. Poor risk features in this index: albumin < 4 g/dL, Hgb < 10.5 g/dL, male sex, age >/= 45, stage IV disease, WBC >15,000, and lymphocytopenia (lymphocyte count < 600 and/or less than 8% of the WBC)
- HAART (either during or immediately following chemotherapy) is essential for any chance at prolonged survival.
AZT should be avoided as it can prolong or exacerbate chemotherapy-induced neutropenia.
ddI and/or d4T can increase incidence of neuropathy.
- In post-HAART era, complete remission rates approach 80%, with overall survival greater than 50%.
- G-CSF routinely used during chemotherapy to shorten duration of neutropenia.
- Standard regimens of ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine) and Stanford V have been used.
- Plans for a collaborative study evaluating Rituxan and ABVD are underway.
- Case reports exploring autologous stem cell transplant for patients with relapsed/refractory disease demonstrate tolerance and efficacy.