|Table of Contents|

Clinical characteristics and prognostic analysis of MLL-AF4 positive acute leukemia patients

Journal Of Modern Oncology[ISSN:1672-4992/CN:61-1415/R]

Issue:
2023 18
Page:
3427-3431
Research Field:
Publishing date:

Info

Title:
Clinical characteristics and prognostic analysis of MLL-AF4 positive acute leukemia patients
Author(s):
ZHANG Meixiang; WANG Chunjian; SHI Lin; LI Yan; REN Hanyun
Department of Hematology,Peking University International Hospital,Beijing 102206,China.
Keywords:
acute leukemia; gene rearrangement; MLL; prognosis; lineage switch
PACS:
R733.71
DOI:
10.3969/j.issn.1672-4992.2023.18.017
Abstract:
Objective:To investigate the clinical features and prognosis of MLL-AF4 positive acute leukemia(AL).Methods:10 adult cases with MLL-AF4 positive AL were analyzed retrospectively and related literatures were reviewed to clarify the clinical featrues and prognosis of MLL-AF4 patients.Results:6 out of the 10 patients were females,with a median age of 34.5 years old.The number of median white blood cells at the time of diagnosis was 114.89×109/L.5 cases complicated with disseminated intravascular coagulation (DIC) and 4 cases with extramedullary invasion.The immunophenotypic characterization of 8 patients was acute B-lymphoblastic leukemia (B-ALL),including 6 case of pro-B-ALL and 2 case of pre-B-ALL.2 patients showed mixed phenotype acute leukemia (MPAL),one displayed a mixture of B/T lymphoid features and the other was B lymphoid/undifferentiated type.9 patients had chromosome t(4;11)(q21;q23).6 of 7 cases with B-ALL achieved the first complete remission(CR) after conventional chemotherapy.4 patients underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) in CR,2 cases died of post-transplantation infection,cerebral hemorrhage,and central recurrence,2 cases gained long term survival.2 patients without transplantation both relapsed within 2 courses of treatment.One patient of 2 patients with MPAL achieved CR after induction chemotherapy.Three relapsed patients were treated with Blinatumomab,one developed extramedullary relapse after CR2,and treatment failed in other two.Lineage switch occurred in 2 B-ALL patients during therapy.Conclusion:Adult MLL-AF4 positive AL has unique clinical characteristics.The majority is pro-B-ALL and few is pre-B-ALL and MPAL，often onset with high white blood cell counts，extramedullary infiltration and coagulable abnormality.Conventional chemotherapy has a higher response rate,but it's easy to relapse and lineage switch.Blinatumomab is effective as a salvage therapy,but allo-HSCT in MRD negative status should have the only potential to improve the prognosis of MLL-AF4+AL.

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