Case Presentation: A 29 year old male with a history of known dental caries and HIV with a CD4 count of 871/uL and an undetectable viral load presented with one month of right-sided jaw pain and facial numbness.
He was initially treated for Bell’s palsy and dental abscess without improvement. It was recommended that he see an oral surgeon, but prior to doing so he returned to the emergency department due to worsening symptoms. He also noted drooling, chills, weight loss, and poor oral intake due to pain. Vitals were Temp 36.7, HR 59, BP 144/88, RR 18, and SpO2 98%. On exam, he had generalized oral erythema, right mandibular retromolar swelling, and multiple missing teeth. Initial labs showed normal CBC and BMP. CT neck with contrast was significant for cervical lymphadenopathy, right upper dental caries, and soft tissue swelling in the right retromolar region. No distinct abscess was present, but the patient was started on ampicillin-sulbactam given concern for oral cellulitis. He had minimal improvement on antibiotics and given his persistent pain, diffuse lymphadenopathy, and history HIV, he was evaluated for lymphoma.
Initial labs showed an LDH of 3008 U/L, uric acid 10.3 mg/dL, and phosphorus 4.9 mg/dL. Due to concern for tumor lysis syndrome he was started on normal saline and allopurinol and given a dose of rasburicase. Otolaryngology took the patient for direct laryngoscopy and excisional lymph node biopsy. Pathology showed the presence of numerous tangible body macrophages imparting a starry sky appearance and FISH results showed c-myc positivity with t(8;14) translocation consistent with a diagnosis of Burkitt lymphoma. PET scan revealed diffuse lymph node involvement, diffuse bone and marrow involvement, and some areas of uptake in the liver. MRI of the brain showed multiple lesions concerning for central nervous system involvement. Flow cytometry of cerebrospinal fluid was consistent with Burkitt lymphoma. The patient was started on the Modified Magrath protocol involving intrathecal methotrexate. He has since completed chemotherapy and has been in remission for the past 9 months.
Discussion: Burkitt lymphoma is a highly aggressive B-Cell Non-Hodgkin lymphoma. It is characterized by a translocation of chromosome 8 resulting in deregulated expression of the MYC oncogene leading to the prevention of apoptosis. It is separated by epidemiologic pattern into 3 categories: endemic, sporadic, and immuno-deficiency related. Immuno-deficiency related Burkitt lymphoma commonly presents in HIV patients with a CD4 count greater than 200/uL and no signs of opportunistic infections. It is important to note that Burkitt lymphoma is the fastest growing human tumor with a cell doubling time of 24-48 hours and rapid diagnosis, staging, and treatment are imperative. Also adding to the urgency is that with high cell turn-over, tumor lysis syndrome can be seen even prior to treatment.
Conclusions: Burkitt lymphoma should always be in the differential diagnosis when evaluating a patient with HIV for head/neck swelling or abdominal mass, especially in a patient with a CD4 count greater than 200/uL.