Mature B cell acute lymphoblastic leukaemia with KMT2A-MLLT3 transcripts in children: three case reports and literature reviews.
Background: Mature B cell acute lymphoblastic leukaemia (BAL) is characterised by French-American-British (FAB)-L3 morphology and the presence of surface immunoglobulin (sIgM) light chain restriction. BAL is also considered as the leukaemic phase of Burkitt lymphoma (BL), in which t (8; 14) (q24; q3...
| Publicado en: | Orphanet Journal of Rare Diseases Vol. 16; no. 1; pp. 1 - 11 |
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| Autores principales: | , , , , |
| Formato: | research Journal Article |
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BioMed Central
7/30/2021
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=151665139&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 151665139 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 17501172 38NP jtl: Orphanet Journal of Rare Diseases issn: 17501172 maglogo: N pubinfo: dt: 7/30/2021 vid: 16 iid: 1 pid: 24147 pub: BioMed Central artinfo: ui: 151665139 151665139 NLM34330316 151665139 10.1186/s13023-021-01972-5 NLM34330316 151665139 ppf: 1 ppct: 10 formats: tig: atl: Mature B cell acute lymphoblastic leukaemia with KMT2A-MLLT3 transcripts in children: three case reports and literature reviews. aug: au: Cui, Yinghui Zhou, Min Zou, Pinli Liao, Xin Xiao, Jianwen affil: Division of Haematology and Oncology, Children's Hospital of Chongqing Medical University, No. 136, Zhongshan 2nd Road, Yuzhong District, 400014, Chongqing, People's Republic of China sug: subj: Leukemia, Lymphocytic, Acute Hematopoietic Stem Cell Transplantation Lymphoma, B-Cell Male Human B Lymphocytes Genes Prospective Studies Female Infant Child Nuclear Proteins Child, Preschool Comparative Studies Multicenter Studies Evaluation Research Validation Studies Clinical Assessment Tools Scales Infant: 1-23 months Child: 6-12 years Child, Preschool: 2-5 years Male Female ab: Background: Mature B cell acute lymphoblastic leukaemia (BAL) is characterised by French-American-British (FAB)-L3 morphology and the presence of surface immunoglobulin (sIgM) light chain restriction. BAL is also considered as the leukaemic phase of Burkitt lymphoma (BL), in which t (8; 14) (q24; q32) or its variants are related to the myelocytomatosis oncogene (MYC) rearrangement (MYCr) is usually present. However, BAL with lysine methyltransferase 2A (KMT2A, previously called Mixed lineage leukaemia, MLL) gene rearrangement (KMT2Ar, previously called MLLr) is rare.Results: Three BAL patients with KMT2Ar were enrolled between January 2017 and November 2019, accounting for 1.37% of the B-ALL population in our hospital. We also reviewed 24 previously reported cases of BAL and KMT2Ar and analysed the features, treatment, and prognosis. Total 13 males and 14 females were enrolled in our research, and the average age at diagnosis was 19.5 ± 4.95 months old. In these 27 patients, renal, central nervous system (CNS) and skin involvement were existent in 6, 4 and 3 patients, respectively; 26 patients (26/27) showed non-ALL-L3 morphology, while one patient is ALL-L3; overexpression of CD19 was detected in most cases, negative or suspicious expression of CD20 was found in 64% of patients. KMT2Ar was reported, but MYCr was not observed. 25 patients (25/27) achieved complete remission after chemotherapy or Stem cell transplantation. The patients were sensitive to chemotherapy, prospective event-free survival (pEFS) of BAL patients with KMT2Ar who received allogeneic haematopoietic stem cell transplantation (allo-HSCT) was higher than that in patients who received chemotherapy alone (83.33% vs 41.91%).Conclusion: BAL patients with KMT2Ar had unique manifestations, including younger age at diagnosis and overexpression of CD19; expression of CD20 was rare, and MYCr was undetectable. The pEFS was higher in patients undergoing allo-HSCT than in patients undergoing chemotherapy alone. pubtype: Academic Journal doctype: research Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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