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

[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.

[学科分类] 生理学

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