Burkitt's non-Hodgkins lymphoma presenting as facial nerve palsy in HIV-positive patients

Abstract

An isolated facial nerve palsy is rare as the presentation of a central nervous system lymphoma. In this case series, we present the clinical features of three HIV-positive patients presenting with facial nerve palsies due to HIV-associated Burkitt's lymphoma. These patients had a non-resolving facial paralysis, which occurred during a late stage of HIV. Magnetic resonance imaging (MRI) did not show leptomeningeal enhancement. Cerebrospinal fluid revealed a lymphocytosis with elevated protein and low glucose levels. The diagnosis of Burkitt's lymphoma was made on histology which showed the characteristic 'starry sky' appearance due to scattered tangible body-laden macrophages. The patients were commenced on the intensive chemotherapy regimen of CODOX-M/IVAC. Two patients died of disease progression and the third patient died of chemotherapy toxicity. This case series highlights the need for a high index of suspicion for underlying malignancy when a patient presents with a persistent facial paralysis in the later stages of HIV infection.

Burkitt non-Hodgkin lymphoma presenting with mental neuropathy (‘numb chin’ syndrome) in an HIV-positive patient

“…The facial nerve is the most commonly involved cranial nerve among HIV-infected patients with Burkitt lymphoma 4,5 and central nervous system involvement, classified as stage IV disease, is associated with a poorer prognosis. Involvement of other cranial nerves is less common, and a small number of reports from the pre-highly active antiretroviral therapy era describe patients with HIV-associated lymphoma and involvement of the trigeminal nerve in the form of mental neuropathy.…”

Aggressive extensive cardiac mass in an HIV-1-infected patient: should we go for comfort therapy?

“…However, due to the advent of HAART, patients with HIV should be approached as in immunocompetent patients. 3,4 Our case illustrates that a pathology specimen is essential in therapeutic strategy planning for a cardiac mass, also if the primary imaging findings look dramatic at the start. Microscopy based decision-making is necessary and can lead to a life-saving therapy, especially in HIV-1-infected patients.…”

“…Studies describe a 10-20% individual lifetime risk of BL for an HIV-infected person, which is unaffected by a combination of highly active antiretroviral therapy (HAART) or CD4 count. 3 Despite the strong epidemiologic evidence linking HIV to the development of BL, the causal pathway remains unknown. 4 BL is a highly aggressive B-cell malignancy associated with the translocation of the c-myc oncogene, which causes high rates of proliferation and cell turnover.…”

“…Since BL carries a high risk of central nervous system (CNS) involvement, attention to CNS prophylaxis with high doses of systemic chemotherapy able to cross the blood-brain barrier, intrathecal therapy, or both is necessary. 3,5 There is no role for radiation therapy. 3 In case of cardiac involvement, monitoring is needed during chemotherapy for signs of arrhythmia since life-threatening arrhythmias may develop during tumor necrosis.…”

“…3,5 There is no role for radiation therapy. 3 In case of cardiac involvement, monitoring is needed during chemotherapy for signs of arrhythmia since life-threatening arrhythmias may develop during tumor necrosis. The advent of rituximab, a monoclonal antibody against the B-cell antigen CD20, has significantly improved outcomes in several B-cell lymphomas, including BL.…”