Burkitt's lymphoma

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ΕΤΥΜΟΛΟΓΙΑ

Named after Irish surgeon, Denis Burkitt, who first observed the disease in 1958 while in Uganda. He published his findings in Nature in 1962.[1]

A rapidly-growing, non-Hodgkin's B-cell lymphoma that has provided much insight into viral-mediated transformation through the effects of the Epstein-Barr virus (EBV). The first and original description was of a disfiguring jaw tumour observed in children during Burkitt's work in Africa. His efforts to study the disease ultimately led to collaboration and the subsequent identification of EBV and its association with the disease.

In developed countries, the disease is less common, affecting mainly children and young adults, but seems to lack the characteristic predilection for the jaw. A further subset of disease is seen in immunosuppressed individuals, particularly those with HIV. The three categories are sometimes distinguished as the endemic, sporadic and immunosuppressed forms respectively, although Burkitt was not keen on the two newer categories. Although EBV is found in virtually all endemic cases, the higher prevalence in Africa is thought to be due to the relative immunosuppression from malaria.

Histological Features

Starry sky appearence of Burkitt's lymphoma.

The tumour is mitotically very active with a short doubling-time. A high rate of cell death accompanies this rapid growth. Consequently, apoptotic and necrotic cells are phagocytosed by macrophages. These macrophages can be seen to contain cellular debris and have a small clearing around them histologically. Tingible body macrophages are normal features of undiseased lymph nodes, but in Burkitt's lymphoma the amount of cell death results in an increased number of tingible body macrophages scattered against a background of dark tumour. The appearance has been likened to a starry sky.

Other high grade lymphomas may give rise to the 'starry sky' appearance and strict diagnostic criteria requires the identification of a characteristic translocation, t(8,14), between the IgH gene and myc proto-oncogene, resulting in overactivity of myc signalling network.

References


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