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The Leukemia/Bone Marrow Transplant Program of BC

Healthcare Professionals
Cancer Management Guidelines

Burkitt Lymphoma and Related Disorders

Updated: May 2016

Diagnosis

Burkitt Lymphoma

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.

Required Tests

  • 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.
  • Lumbar puncture

Staging

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.

Stage

Involvement

1

Single lymph node region (1) or one extralymphatic site (1E).

2

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)

3

Lymph node regions on both sides of diaphragm (3) which may be accompanied by local extralymphatic extension (3E)

4

Diffuse involvement of one or more extralymphatic organs or sites

Symptoms

A =

no B symptoms

B =

presence of at least one of these:

 

1

unexplained weight loss > 10% baseline during 6 months prior to staging

 

2

unexplained fever > 38oC/font>

 

3

night sweats

Prognosis

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.

Required Tests

Same as Burkitt Lymphoma

Staging

Same as Burkitt Lymphoma

Prognosis

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.

Treatment

Algorithms

Click images to enlarge.

Burkitt Lymphoma Treatment Algorithm Double Hit Lymphoma Referral Algorithm

Protocols

BCCA Chemotherapy Protocols and PPOs

Publications

  1. Mead GM, Barrans SL, Qian W, Walewski J, Radford JA, Wolf M, Clawson SM, Stenning SP, Yule CL, Jack AS. A prospective clinicopathologic study of dose-modified CODOX-M/IVAC in patients with sporadic Burkitt lymphoma defined using cytogenetic and immunophenotypic criteria (MRC/NCRI LY10 trial). Blood 112:2248-2260, 2008.
  2. Song KW, Barnett MJ, Gascoyne RD, Horsman DE, Forrest DL, Hogge DE, Lavoie JC, Nantel SH, Nevill TJ, Shepherd JD, Smith CA, Sutherland HJ, Voss NJ, Toze CL and Connors JM. Haematopoietic stem cell transplantation as primary therapy of sporadic adult Burkitt lymphoma. Br J Haem 133:634-637, 2006.
  3. Petrich AM, Gandhi M, Jovanovic B, Castillo JJ, Rajguru S, Yang DT, Shah KA, Whyman JD, Lansigan F, Hernandez-Ilizaliturri FJ, Lee LX, Barta SK, Melinamani S, Karmali R, Adeimy C, Smith S, Dalal N, Nabhan C, Peace D, Vose J, Evens AM, Shah N, Fenske TS, Zelenetz AD, Landsburg DJ, Howlett C, Mato A, Jaglal M, Chavez JC, Tsai JP, Reddy N, Li S, Handler C, Flowers CR, Cohen JB, Blum KA, Song K, Sun HL, Press O, Cassaday R, Jaso J, Medeiros LJ, Sohani AR, Abramson JS. Impact of induction regimen and stem cell transplantation on outcomes in double-hit lymphoma: a multicenter retrospective analysis. Blood 124(15):2354-61, 2014

 

The information contained in these guidelines is a statement of consensus of Leukemia/BMT Program of BC professionals regarding their views of currently accepted approaches to treatment. Any clinician seeking to apply or consult these documents is expected to use independent medical judgment in the context of individual clinical circumstances to determine any patientís care or treatment. Use of these guidelines and documents is at your own risk and is subject to the Leukemia/BMT Program of BCís terms of use available at Terms of Use.

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