Burkitt lymphoma (BL)

​

Burkitt lymphoma (BL), a highly aggressive B-cell lymphoma, represents approximately 2.5% of all non-Hodgkin lymphomas (NHLs). BL preferentially involves extranodal sites, such as the small intestine or jaw, or may manifest as acute leukemia. The genetic hallmark of BL is a reciprocal translocation of the MYC gene on chromosome 8 most commonly with IGH gene or, alternatively, with the κ or λ immunoglobulin light chain genes.

The diagnosis of BL is based on a combination of morphologic, immunophenotypic, and cytogenetic findings. The distinction between BL and other high-grade B-cell lymphomas is clinically important, not only because BL is a tumor of extremely high proliferation rate but because patients are at special risk for central nervous system involvement. The morphologic features of BL—sheets of monomorphic medium-sized B cells with basophilic cytoplasm, numerous mitotic figures, and admixed macrophages (“starry-sky pattern”)—are not specific because lymphoblastic lymphoma, diffuse large B-cell lymphoma (DLBCL), plasmablastic lymphoma, and even a high-grade T-cell lymphoma can have this picture.

​

Immunophenotype:

Evaluation of the tumor immunophenotype might help to differentiate BL from these entities. The characteristic immunophenotype of BL is a mature B cell with germinal center cell differentiation. The tumor cells are positive for B cell–associated antigens such as CD19, CD20, CD22, and CD79a and express surface immunoglobulins (SIgs) with light chain restriction and the germinal center cell markers CD10 and bcl-6. The tumor cells are negative for CD5, CD23, and terminal deoxynucleotidyl transferase (TdT) and usually negative for bcl-2; however, bcl-2 can be expressed in 10% to 20% of cases.

​

                                                                                                 CASE -1

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Case 1 showed several immunophenotypic deviations in addition to lack of SIg light chain expression. Case 1 have  expression of  B cell–associated antigens, CD19, CD20, CD22 and CD79a, by flow cytometry. Case 1 lack the expression of  CD10, CD15, NG2, CD3, cCD3, MPO, CD13, CD33 and CD7. they also expressed the CD38, CD123, CD58, CD81 and HLA-DR.

 

                                                                                                    CASE -2

 

 

 

 

 

 

 

 

 

 

 

 

 

 

​

​

​

​

​

​

​

​

 

 

 

 

 

 

Case 2  have  expression of sIgM with  B cell–associated antigens, CD19, CD20, CD10 and CD79a, by flow cytometry. Case 2 lack the expression of  CD13, CD34, MPO, CD13. they also expressed the HLA-DR.

 

​

                                                                                                    CASE -3

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Case 3 showed  SIg light chain expression with  B cell–associated antigens, CD19, CD20, CD22,CD79a and Kappa lambda, by flow cytometry. Case 3 lack the expression of  CD10, MPO, CD13, cCD3, CD33 and CD34. they also expressed the CD38.

​

Reference:

​

• Burkitt D. A sarcoma involving the jaws in African children. Br J Surg 1958;46:218–223. Google Scholar

• Burkitt DP. The discovery of Burkitt’s lymphoma. Cancer 1983;51:1777–1786.Google Scholar

• Burkitt D, Wright D. Burkitt’s lymphoma, First Edition. Edinburgh and London: E and S Livingstone, 1970:1–251.Google Scholar

• Van den Bosch CA. Is endemic Burkitt’s lymphoma an alliance between three infections and a tumour promoter? Lancet Oncol 2004;5:738–746.

  CrossRefMedlineGoogle Scholar

• Diebold J. Burkitt lymphoma. In: Jaffe E, Harris N, Stein H et al., eds. Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues. Washington, DC: IARC Press, 2001:181–184.Google Scholar

• Blum KA, Lozanski G, Byrd JC. Adult Burkitt leukemia and lymphoma. Blood 2004;104:3009–3020.Abstract/FREE Full Text

• Boerma EG, van Imhoff GW, Appel IM et al. Gender and age-related differences in Burkitt lymphoma--epidemiological and clinical data from The Netherlands. Eur J Cancer 2004;40:2781–2787.Google Scholar

• Burmeister T, Schwartz S, Horst HA et al. Molecular heterogeneity of sporadic adult Burkitt-type leukemia/lymphoma as revealed by PCR and cytogenetics: correlation with morphology, immunology and clinical features. Leukemia 2005;19:1391–1398.Google Scholar

• Gong JZ, Stenzel TT, Bennett ER et al. Burkitt lymphoma arising in organ transplant recipients: a clinicopathologic study of five cases. Am J Surg Pathol 2003;27:818–827.Google Scholar

• Xicoy B, Ribera JM, Esteve J et al. Post-transplant Burkitt’s leukemia or lymphoma. Study of five cases treated with specific intensive therapy (PETHEMA ALL-3/97 trial). Leuk Lymphoma 2003;44:1541–1543.Google Scholar

• Doll DC, List AF. Burkitt’s lymphoma in a homosexual. Lancet 1982;1:1026–1027.Google Scholar

• Ziegler JL, Drew WL, Miner RC et al. Outbreak of Burkitt’s-like lymphoma in homosexual men. Lancet 1982;2:631–633.Google Scholar

• Rodig SJ, Vergilio JA, Shahsafaei A, et al. Characteristic expression patterns of TCL1, CD38, and CD44 identify aggressive lymphomas harboring a MYC translocation. Am J Surg Pathol. 2008;32:113-122.

• Chuang SS, Ye H, Du MQ, et al. Histopathology and immunohistochemistry in distinguishing Burkitt lymphoma from diffuse large B-cell lymphoma with very high proliferation index and with or without a starry-sky pattern: a comparative study with EBER and FISH. Am J Clin Pathol. 2007;128:558-564.

• Shaffer LG, Slovak ML, Campbell LJ, eds. An International System for Human Cytogenetic Nomenclature. Basel, Switzerland: Karger; 2009.

• Picker LJ, Weiss LM, Medeiros LJ, et al. Immunophenotypic criteria for the diagnosis of non-Hodgkin’s lymphoma. Am J Pathol. 1987;128:181-201.

• Liendo C, Danieu L, Al-Katib A, et al. Phenotypic analysis by flow cytometry of surface immunoglobulin light chains and B and T cell antigens in lymph nodes involved withnon-Hodgkin’s lymphoma. Am J Med. 1985;79:445-454.

• de Martini RM, Turner RR, Boone DC, et al. Lymphocyte immunophenotyping of B-cell lymphomas: a flow cytometric analysis of neoplastic and nonneoplastic cells in 271 cases. Clin Immunol Immunopathol. 1988;49:365-379.

• Batata A, Shen B. Diagnostic value of clonality of surface immunoglobulin light and heavy chains in malignant lymphoproliferative disorders. Am J Hematol. 1993;43:265-270.

• Geary WA, Frierson HF, Innes DJ, et al. Quantitative criteria for clonality in the diagnosis of B-cell non-Hodgkin’s lymphoma by flow cytometry. Mod Pathol. 1993;6:155 161.

• Fukushima PI, Nguyen PK, O’Grady P, et al. Flow cytometric analysis of kappa and lambda light chain expression in evaluation of specimens for B-cell neoplasia.Cytometry. 1996;26:243-252