Diagnosis>Malignancies>

PATHOGENS

• Almost always EBV-associated, unlike in HIV-negative pts.

CLINICAL

• 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).

DIAGNOSIS

• 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.

TREATMENT

• 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.

REFERENCES

REFERENCED WITHIN THIS GUIDE

• Lymphoma, Non-Hodgkins (NHL)
• Neutropenia