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Kohla S, Ibrahim FA, Mudawi D, et al. High-Grade Epstein-Barr Virus-Negative Biphenotypic Lymphoma with Expression of B- and T-Cell Markers and Leukemia Presentation: Case Report and Literature Review. Case Rep Oncol. 2020;13(3):1215-1226doi: 10.1159/000510403.
Kohla, S., Ibrahim, F. A., Mudawi, D., Akiki, S., Soliman, D., Al-Sabbagh, A., Youssef, R. R. H., & Yassin, M. A. (2020). High-Grade Epstein-Barr Virus-Negative Biphenotypic Lymphoma with Expression of B- and T-Cell Markers and Leukemia Presentation: Case Report and Literature Review. Case reports in oncology, 13(3), 1215-1226. https://doi.org/10.1159/000510403
Kohla, Samah, et al. "High-Grade Epstein-Barr Virus-Negative Biphenotypic Lymphoma with Expression of B- and T-Cell Markers and Leukemia Presentation: Case Report and Literature Review." Case reports in oncology vol. 13,3 (2020): 1215-1226. doi: https://doi.org/10.1159/000510403
Kohla S, Ibrahim FA, Mudawi D, Akiki S, Soliman D, Al-Sabbagh A, Youssef RRH, Yassin MA. High-Grade Epstein-Barr Virus-Negative Biphenotypic Lymphoma with Expression of B- and T-Cell Markers and Leukemia Presentation: Case Report and Literature Review. Case Rep Oncol. 2020 Sep 30;13(3):1215-1226. doi: 10.1159/000510403. eCollection 2020. PMID: 33173488; PMCID: PMC7590753.
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Case Rep Oncol. 2020 Sep 30;13(3):1215-1226. doi: 10.1159/000510403. eCollection 2020.

High-Grade Epstein-Barr Virus-Negative Biphenotypic Lymphoma with Expression of B- and T-Cell Markers and Leukemia Presentation: Case Report and Literature Review.

Case reports in oncology

Samah Kohla, Feryal A Ibrahim, Deena Mudawi, Susanna Akiki, Dina Soliman, Ahmad Al-Sabbagh, Reda R H Youssef, Mohamed A Yassin

Affiliations

  1. Department of Lab Medicine and Pathology, Hematology Division, Hamad Medical Corporation, Doha, Qatar.
  2. Department of Clinical Pathology, Hematology Division, Faculty of Medicine, Al-Azhar University, Cairo, Egypt.
  3. Department of Hematology-Oncology, Hamad Medical Corporation, Doha, Qatar.
  4. Department of Lab Medicine and Pathology, Molecular and Cytogenetic Division, Hamad Medical Corporation, Doha, Qatar.
  5. Department of Clinical Imaging, Hamad Medical Corporation, Doha, Qatar.

PMID: 33173488 PMCID: PMC7590753 DOI: 10.1159/000510403

Abstract

Lymphomas are presently categorized according to their origin from B or T lymphocytes. The co-expression of CD3 in B-cell lymphomas or CD20 in T-cell lymphomas has been rarely reported. Immature and less often mature lymphomas may incorporate the rearrangements of both B- and T-cell antigen receptor genes (dual genotype or bigenotype). Lymphoma cells with a sole genotype hardly concurrently express both B- and T-cell markers (biphenotypic lymphomas). We describe a 63-year-old female who was presented with obstructive jaundice and epigastric pain of 10 days. Initial CBC revealed 43×103/μL white blood cells, 11.2 g/dL hemoglobin, and 88x103/μL platelets. CT abdomen revealed hepatomegaly and suspected pancreatic mass with large retroperitoneal lymph nodal mass. Peripheral smear showed 56% lymphoid cells with blast morphology. The bone marrow (BM) aspirate smear was infiltrated by 83% immature-looking cells. BM biopsy showed interstitial to diffuse extensive infiltration by primitive-looking cells, positive for pan-B-cell antigens CD20, CD79, and PAX5 as well as the T-cell antigen CD4, CD5, CD3, while negative for all immaturity markers (CD34, TdT, and CD1a). In situ hybridization for Epstein-Barr virus (EBV)-encoded small RNA (EBER) was negative. Flow cytometry on BM aspirate showed an abnormal population (50%) expressing the B-cell antigens (CD19, CD20, CD79, CD22) and CD10, and showed lambda light chain restriction as well as the T-cell antigens cCD3 and CD4 with partial CD5. The analysis showed, also, another abnormal population of lambda restricted monotypic B cells (8%) with dimmer CD45 (blast gate) and showed the same immunophenotype (expressing the B-cell antigens), but negative for CD10, cCD3, CD5, and CD4. Conventional cytogenetic revealed complex karyotype. Molecular studies revealed rearrangements of the immunoglobulin heavy chain region consistent with a clonal B-cell population. TCR gene rearrangement analysis was equivocal concerning clonality but was not conclusive for clonal T-cell disease. Our final diagnosis was peripheral blood and BM involvement by EBV-negative high-grade lymphoid neoplasm (in leukemic phase with blast morphology) and an ambiguous immunophenotype with a differential diagnosis that may include the rare entity of bigenotypic lymphoma or an unusual case of high-grade B-cell lymphoma with aberrant expression of T-cell markers (biphenotypic lymphomas).

Copyright © 2020 by S. Karger AG, Basel.

Keywords: Bigenotypic lymphoma; Biphenotypic lymphoma; CD3-positive B-cell lymphoma; Epstein-Barr virus-negative lymphoma; High-grade lymphoma; Lymphoma

Conflict of interest statement

The authors declare that they have no relevant financial interests.

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