B-Cell Precursor Acute Lymphoblastic Leukaemia/Lymphoma with Myc and Bcl-2 Rearrangements, a Case Report of an Unclassified “New” Entity

Journal of Case Reports

article

Roberto Secchi¹  Federico Meconi¹  Fabiana Esposito¹  Annagiulia Zizzari¹  Vito Mario Rapisarda¹  Ida Provenzano¹  Manuela Rizzo¹  Lucia Anemona¹  Maria Cantonetti² 

[1] Department of Biomedicine and Prevention, Haematology Division;Anatomic Pathology, Department of Biomedicine and Prevention, University of Tor Vergata

关键词: B-Lymphocytes;    Cytogenetic Analysis;    Leukemia;    Lymphoma;    Prognosis.;   

DOI  :  10.17659/01.2021.0001

学科分类：生理学

来源: Journal of Case Reports

【 摘 要 】

Background: Lymphomas with Myc and Bcl2 and/or Bcl6 rearrangements, commonly defined as Double hit Lymphomas (DHI) or Triple hit Lymphomas (THI) have a poor prognosis than those with diffuse large B-cell lymphoma (DLBCL) NOS. Combined Myc and Bcl-2 and Bcl-6 rearrangements are reported in rare cases of follicular lymphoma (FL) and acute lymphoblastic B-cell precursor leukemia/lymphoma (BCP-ALL). Case Report: Here in, we report a case of a fifty year-old man, who was referred to our hospital after being diagnosed with de novo DH-BCP-ALL, presenting with both diffuse cranial and spinal meningeal involvement and multiple other extra-nodal localizations (cutaneous, testicular, gastro-enteric and skeletal), in addition to nodal localizations such as para-cardiac and para-renal. After reaching Partial Response (PR) with first line treatment, the patient early relapsed and was refractory to new lines of therapy with an overall survival (OS) of 7 months. Conclusion: BCP-ALL with combination of Myc and Bcl-2 and/or Bcl-6 rearrangements named “De novo DH-BCP-ALLs” have to be classified as a separate entity although they are rare and their clinic immune-phenotypic and cytogenetic features are not well characterized.

【 授权许可】

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