Abstract
Anaplastic lymphoma kinase (ALK)- negative anaplastic large cell lymphoma (ALCL) is an aggressive CD30-positive non- Hodgkin lymphoma. ALK-ALCL rarely manifests with extensive bone marrow and peripheral blood involvement (known as “leukemic phase”). A 54-year-old woman was diagnosed with ALK-ALCL in leukemic phase, characterized by an extremely poor prognosis. Lymphoma cells in this case showed chromosomal translocation 1p36.1- encoded RUNX3 and overexpression of its protein. She was refractory to CHOP and salvage chemotherapy. Fortunately, she achieved complete remission with three cycles of Brentuximab vedotin (BV) and underwent umbilical cord blood transplantation. However, she died due to treatment-related mortality on day 129. The autopsy findings showed no lymphoma cells. Treatment strategy for ALK-ALCL is controversial, but the efficacy of BV in CD30-positive peripheral T-cell lymphoma not only as salvage regimens, but also in first line, has been reported in recent years. BV may be an effective option for ALK-ALCL in leukemic phase.
Highlights
Anaplastic lymphoma kinase (ALK)negative anaplastic large cell lymphoma (ALCL) is a CD30-expressing aggressive Tcell lymphoma that has been entered as a provisional entity in the WHO 2008 classification representing 2 to 3% of nonHodgkin lymphomas (NHLs) and 12% of T-cell NHLs.[1]
Brentuximab vedotin (BV) is an antibody-drug conjugate composed of an anti-CD30 chimeric antibody conjugated with the microtubule-disrupting agent, monomethyl auristatin E (MMAE)
Like the surface marker in our patient, the expression of CD56 in ALK-positive and ALK-negative ALCL is associated with a higher incidence of bone marrow involvement and is considered a poor prognostic factor for overall survival (OS).[7]
Summary
Anaplastic lymphoma kinase (ALK)negative anaplastic large cell lymphoma (ALCL) is a CD30-expressing aggressive Tcell lymphoma that has been entered as a provisional entity in the WHO 2008 classification representing 2 to 3% of nonHodgkin lymphomas (NHLs) and 12% of T-cell NHLs.[1]. BV efficacy in relapsed or refractory ALCL has been recently reported to have favorable outcomes.[4] is reported the case of a refractory ALK-ALCL patient in leukemic phase. In this case, the patient achieved complete remission (CR) with single agent BV therapy, and could undergo umbilical cord blood transplantation. The patient achieved complete remission (CR) with single agent BV therapy, and could undergo umbilical cord blood transplantation This case indicates that BV monotherapy could act as an effective bridge to allogeneic hematopoietic stem cell transplantation (allo-HSCT), despite refractoriness to the three preceding regimens
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