Burkitt lymphoma

Burkitt lymphoma is an aggressive B-cell lymphoma predominantly affecting children.

Epidemiology

Burkitt lymphoma is the most common (40%) type of non-Hodgkin lymphoma in childhood. Median age is eight years with a male predominance (M:F = 4:1) . It is less common in adults, accounting for 1-2% of lymphomas .

It is considered endemic in parts of Africa where rates are up to 50 times higher than in the USA. Cases occur sporadically outside of the tropics.

Risk factors
  • HIV/AIDS
  • post-transplant immunosuppression

Clinical presentation

Burkitt lymphoma affects many organs, and this affects presentation. Extranodal involvement is common (~30%) at presentation, most often presenting as an abdominal or pelvic mass. Most patients present with widespread disease.

Pathology

Three forms of Burkitt lymphoma have been described :

  • endemic Burkitt lymphoma: linked to Epstein-Barr virus (EBV) and Plasmodium falciparum malaria infections
  • sporadic Burkitt lymphoma: etiology unknown
  • immunodeficiency-associated Burkitt lymphoma: occurs in patients with HIV, post-transplant or congenital immunosuppression

Burkitt lymphoma is an aggressive tumor with a doubling time of 24 hours. It can present in a wide variety of locations:

  • head and neck, e.g. facial bones, Waldeyer ring
  • pleural space (~70%)
  • gastrointestinal tract, especially the ileocecal region
  • mesentery, peritoneum, retroperitoneum
  • kidneys
  • gonads (~75%)

Nodal involvement is more common in adults than in children .

Radiographic features

Radiographic features vary widely depending on organ involvement:

  • abdominal manifestations of Burkitt lymphoma
  • musculoskeletal manifestations of Burkitt lymphoma
  • head and neck manifestations of Burkitt lymphoma

Treatment and prognosis

Burkitt lymphoma can be treated with chemotherapy. In children, the prognosis is good with survival rates >90%. In adults, the prognosis is poorer, with a 5-year survival rate of ~50% and is even worse with bone marrow or CNS involvement (>30% 5-year survival rate) .

History and etymology

First described by Denis Parsons Burkitt (1911-1993), an Irish surgeon, in 1958 in Uganda, Africa .

Differential diagnosis

  • other forms of high-grade B-cell lymphoma
  • Crohn disease may mimic Burkitt lymphoma on ultrasound, barium studies, and CT