Burkitt lymphoma is an uncommon non-Hodgkin’s lymphoma. It is a B-cell lymphoma with an extremely short doubling time and often presents with extranodal sites. Characteristic cytogenetic changes lead to deregulation of the MYC gene. This lymphoma requires rapid assessment and treatment.In British Columbia adult patients with an established or probable diagnosis of Burkitt lymphoma should be referred to the Leukemia/BMT Program at the Vancouver General Hospital. Children should be referred to the BC Children’s Hospital.
Lymph node/tissue biopsy;
CBC and differential
Electrolytes, BUN, creatinine, uric acid, liver function tests, LDH
INR, PTT and fibrinogen
Bone marrow aspirate and biopsy with cytogenetic analysis and flow cytometry for immunophenotyping
Computed tomography (CT) scan of the neck, chest abdomen and pelvis. CT of the head should also be considered, especially if there are CNS symptoms.
The stage of disease is of major therapeutic and prognostic significance. The staging system used is based on the Ann Arbor system with additional consideration of the bulk or size of individual tumours. The formal stage is assigned using the following system.
Single lymph node region (1) or one extralymphatic site (1E).
Two or more lymph node regions, same side of the diaphragm (2) or local extralymphatic extension plus one or more lymph node regions same side of the diaphragm (2E)
Lymph node regions on both sides of diaphragm (3) which may be accompanied by local extralymphatic extension (3E)
Diffuse involvement of one or more extralymphatic organs or sites
no B symptoms
presence of at least one of these:
unexplained weight loss > 10% baseline during 6 months prior to staging
unexplained fever > 38oC/font>
Patients diagnosed with Burkitt lymphoma can have a 5-year disease free survival rate of 60-80%. The prognosis is dependant upon the stage of disease and the age of the patient.
Double Hit Lymphoma
Double hit lymphomas typically contain a MYC/8q24 breakpoint in combination with a BCL2/18q21 breakpoint. The partner of the BCL2/18q21 breakpoint mostly is the IGH locus at 14q32. The abnormalities are typically associated with the B-cell lymphoma, Unclassifiable, with Features intermediate between Diffuse Large B-cell Lymphoma and Burkitt Lymphoma (BCLU) as well as the Diffuse large B-cell Lymphoma histology. This diagnosis is dependent upon demonstration of the typical cytogenetic changes. Patients who have immunohistochemical evidence of upregulation of the MYC oncogene and the BCL2 oncogene expression but without cytogenetic evidence of the rearrangements are not considered to have true Double Hit Lymphoma.
Same as Burkitt Lymphoma
Same as Burkitt Lymphoma
Patients diagnosed with Double Hit Lymphoma do significantly worse than patients with Burkitt Lymphoma. Prognosis has been found to be influenced by the histology, stage, CNS involvement and age of the patient.
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