Burkitt`s lymphoma with Particular reference to the disease in Sudan

Abstract

During his clinical practice as a general surgeon in Uganda, Dr Denis Burkitt was asked in 1957 to see a paediatric patient with an unusual tumour involving the jaw and soon after another child with the same condition was referred to him. The tumour was rapidly growing and the children died within few weeks. Burkitt recognized that this unusual condition was possibly a previously undescribed malignant tumour. He contacted a large number of hospitals and institutions in Africa including the senior authors and asked us to look for this tumour in Sudan. Burkitt undertook a geographical survey of the incidence of the disease and found it to be correlated with the same temperature and rainfall zones as malaria. This suggested that the tumour might be caused by an infective agent that was linked with the distribution of certain insect carriers. In 1958, he published his first paper on this tumour with his colleagues(1). This survey is regarded as one of the pioneering studies of geographical pathology. At first, the tumour was described as multicentric sarcoma of the jaw. Later it was found to be a lymphoma and was named Burkitt’s lymphoma (BL). It was strongly suspected to be caused by Epstein Barr virus. Clinical features and classification According to the World Health Organization, BL is classified into three main types: endemic, sporadic and HIV associated. Endemic BL affects children in the age group 4-7 years with a male to female ratio of 2:1. Facial bones, particularly the jaws are affected but other parts of the body such as the thyroid, breast, kidneys, gastrointestinal tract and ovaries may be affected. The type described in Uganda and other African countries is the endemic form(2). There is evidence that this form is changing in at least some African countries. Sporadic BL involving mainly other organs apart from the jaws is described in Europe, North America and Japan. A form associated with HIV/AIDS infection has also been described(3). Lynch and El-Hassan were the first to report Burkitt’s lymphoma in Sudan under the title multicentric sarcoma of the jaw4. El-Hassan described another six cases of BL of the jaws among 113 malignant tumors in children5. Nabri described the lymphoma in Equatorial Province and observed that it usually affects the jaws and other organs but jaw involvement was most common(6,7). During the period 1962-73, twenty-nine cases of Burkitt’s lymphoma were described in Sudan; these formed 20% of cases of childhood lymphomas(8). The tumour, mainly of the endemic type, occurred mainly in the southern and western parts of the Sudan where malaria is hyperendemic. We now believe that the pattern of Burkitt’s lymphoma is changing. In the last seven years in our laboratory, patients with this lymphoma were found to be of the sporadic type with involvement of the gastrointestinal tract and retroperitoneum rather than the jaws. The causes for this change are at present the subject of investigation. Central nervous system involvement in BL One of the complications of BL is involvement of the central nervous system. This is difficult to treat and carries a poor prognosis. Of 77 cases of BL in Uganda, 35 patients had neurological changes. These changes included paraplegia, cranial neuropathy, altered level of consciousness and malignant pleocytosis. Paraplegia was a common presenting symptom in BL and responded well to chemotherapy. The association of cranial nerve involvement and pleocytosis with facial bone lesions was believed to be due to the direct extension of the tumour to the dura and arachnoid. Intrathecal chemotherapy was effective in reversing the pleocytosis. Cranial nerve involvement required systemic chemotherapy(