Abstract
BackgroundAIDS-related primary central nervous system lymphoma (AR-PCNSL) is an AIDS-defining disease that usually occurs when the CD4 count is less than 50 cells/μl. The frequency of the disease has substantially decreased in the era of highly active antiretroviral therapy (HAART). Prognosis is poor with rapid progression leading to death within 2–3 months if left untreated.Case descriptionA 65 years old male presented to medical attention with gait disturbance, weight loss and slight left-sided hemiparesis. Human immunodeficiency virus infection was diagnosed with an initial CD4 count of 116 cells/µl and a viral load of 260,000 copies/ml. Magnetic resonance imaging of the brain revealed three brain lesions involving the right frontal lobe and the left parietal lobe, which on biopsy led to a diagnosis of AR-PCNSL. HAART was initiated with whole-brain radiotherapy (WBRT), and the patient declined systemic chemotherapy. Due to poor performance status, he was transferred to palliative care. Under HAART, he slowly recovered with normalization of CD4 count and undetectable viral load. Medical imaging showed complete remission (CR) of the brain lesions. At 3-year follow-up, the patient remains in CR, but presented mild neurocognitive dysfunction possibly secondary to WBRT.ConclusionNowadays, treatment paradigm parallels that of primary central nervous system lymphoma in the immunocompetent population based on systemic chemotherapy (primarily high-dose intravenous methotrexate and steroids) in association with HAART. The role of WBRT is questionable because of late neurotoxic effects.
Highlights
acquired immune deficiency syndrome (AIDS)-related primary central nervous system lymphoma (AR-Primary central nervous system lymphoma (PCNSL)) is an AIDS-defining disease that usually occurs when the CD4 count is less than 50 cells/μl
Nowadays, treatment paradigm parallels that of primary central nervous system lymphoma in the immunocompetent population based on systemic chemotherapy in association with highly active antiretroviral therapy (HAART)
The AIDS epidemic during the early 1990s led to a high incidence of PCNSL with 47.2% of CNS lymphomas occurring in AIDS-patients [8]
Summary
Optimal therapeutic approach for ARPCNSL remains unclear due to the rarity of the disease and the paucity of prospective studies to guide clinical management. WBRT long stood as the gold standard therapy but its potential long-term consequences on neurologic and cognitive function are important features to consider. Treatment modalities are based on systemic chemotherapies that parallel the treatment of PCNSL in the general population, in association with HAART. Several reports showed successful treatment with HAART alone and that it can provide long-term survival even in patients with advanced HIV disease. Complementary treatment should be guided by performance
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