A Burkitt's Lymphoma Case from Nigeria

O.B. is an 11-year-old boy referred from a private clinic to the Department of Pediatrics, University College Hospital (UCH), Ibadan, Nigeria, with a month history of swelling of the left jaw. The left upper first premolar tooth was lost a week prior to presentation. Because the gum was bleeding so easily, the patient had changed from using a toothbrush to foam for cleaning his teeth. There was no weight loss.

Mother had been applying 'Robb' liniment and hot fomentation to the swelling because she perceived it was due to trauma. However, the swelling continued to increase rapidly in size. Out of frustration, the mother visited a ‘native’ doctor who made scarification marks on the swelling to ward off the evil spirit thought to be responsible for the illness. In addition to this, native herbs (name unknown) were applied to the swelling.

As there was no improvement, the patient was taken subsequently to two private hospitals. He was given an intramuscular injection for five days in the first hospital, while on reaching the second hospital, he was promptly referred to UCH, Ibadan.

At the Hospital

The patient was the first of four children in a monogamous family of low socioeconomic status. Physical examination revealed a malnourished boy, weight 25kg (70% of weight for age). There was a smooth mass over the left maxilla and mandible, measuring 9 x 8 cm in the transverse and longitudinal diameter, respectively. The swelling was hard and many scarification marks were visible. There was no intra-oral extension of the mass. The first left upper premolar was missing, but no other tooth was loose. Mild dental mal-alignment was noted. A diagnosis of Burkitt’s lymphoma, stage B, was made.

Jaw radiograph showed loss of the dental lamina dura (a hard layer of bone surrounding the tooth) and marked displacement of teeth. Fine needle aspiration cytology was suggestive of a non-Hodgkin’s lymphoma, Burkitt’s type. On the 16th day of admission, and with the help of the social worker, some of the required cytotoxic drugs were purchased. However, the patient had deteriorated as shown by increased liver size (5cm) and the presence of meningism, suggesting involvement of the cerebrospinal fluid (CSF). A spinal tap was done, and the CSF was shown to have a low glucose level with elevated protein (140mg/dl) but no tumor cells were observed. The chemotherapeutic regimen included: cytarabine at 50mg/m² (45mg), 12 hourly for 6 doses, intrathecal cytarabine (delivered by spinal tap), 36mg/m² (35mg) on days 1 and 5, intravenous (i.v.) cyclophosphamide 1000mg/m² (960mg) on day 1, i.v. oncovin 1.5mg m² (1.4mg) on day 1, and oral prednisolone 40mg daily for five days (COAP). Allopurinol, at a dose of 100mg thrice daily, and liberal oral fluids (3L/day) were administered.

By the fifth day of the first course of chemotherapy, the meningism had resolved. The electrolyte and urea, calcium and phosphate, creatinine and uric acid values were normal except for a low serum sodium level of 126mmol/L.

Between the 23rd and the 31st day of admission the patient developed headache, blurring of vision and occasional dizziness with a left facial nerve palsy and left hemiparesis. Repeat CSF cytology showed clusters of large lymphoblastic cells, indicative of central nervous system involvement by Burkitt’s lymphoma. There was also papillodema, suggesting raised intracranial pressure. The patient became confused on the 31st day of admission. With further financial support provided by the social worker, the second course of treatment was started and 20% mannitol 1gm/kg / dose 6 hourly was given on three occasions in an attempt to control the elevated intracranial pressure. It is interesting to note that the jaw swelling had become reduced in size.

The Loss

On the 37th day of admission the patient had a tonic-clonic seizure lasting about two minutes. The electrolytes were normal. However, the papilloedema had not resolved, suggesting that the raised intracranial pressure caused the seizure. Further seizures ensued, which failed to respond to paraldehyde and phenobarbitone. The patient went into coma after four days and died the following day.

Post mortem showed multiple tumor nodules in both kidneys, leptomeningeal (coverings of the brain and spinal cord) infiltration by the tumor with evidence of brain swelling and left testicular involvement.

submitted by Dr Goke Falade
Ibadan University Hospital
Nigeria