RARE TUMOURS

ABSTRACT: BACKGROUND: Epstein-Barr Virus (EBV) associated lymphoproliferative disorders are encountered in immunodeficiency states. Amongst these, primary CNS lymphoma in HIV/AIDS patients is relatively rare and represents a therapeutic challenge. MATERIALS AND METHODS: This report was generated from patient's medical records throughout hospitalizations and outpatient visits. RESULTS: A 20-year-old female with congenitally acquired HIV and poor medication compliance presented with severe headaches and mild cranial nerve deficits. HIV viral load at presentation was 89,976/ml with CD4 count of 4/uL. Head MRI revealed 3-cm thick-walled ring-enhancing lesion in right basal ganglia. Serum titers for toxoplasmosis were negative. EBV DNA was detected by PCR in CSF without peripheral viremia. Imaging with thallium scan and MRI findings in combination with EBV PCR positive CSF allowed for the presumptive diagnosis of EBV-associated primary CNS lymphoma. Highly active anti-retroviral therapy (HAART) was reinitiated. Karnofsky score was 90%, thus chemotherapy was offered to optimize chance of cure. Agents of choice were high-dose Methotrexate for CNS penetration/anti-lymphoma activity and Rituximab. Within the next 5 months she received 8 cycles of alternating Methotrexate (4000 mg/m2/dose on Day 1) and Rituximab (375 mg/m2/dose on Day 8). Supportive care included leucovorin rescue and urine alkalinization. Therapy was tolerated well without sepsis episodes during neutropenia. Her headaches and neurological deficits resolved after the first cycle of therapy. Serial imaging demonstrated substantial and continuous regression of the lymphoma lesion. No adjunctive radiotherapy was administered. Patient remains in remission 18 months after diagnosis, despite fluctuating viral loads and CD4 counts due to the poor HAART compliance. No chronic hypogammaglobulinemia resulted from therapy to date. CONCLUSION: Given the observed results and acceptable toxicity profile, methotrexate and rituximab combination may be effective and should be considered in addition to HAART for treating EBV-associated CNS lymphoma in young patients with minimal comorbidities and underlying HIV/AIDS.

SUBMITTER: Panosyan E 

PROVIDER: S-EPMC4046297 | biostudies-literature | 2014 Jun

REPOSITORIES: biostudies-literature