- Author:
Li'na WANG
1
;
Yazhen QIN
1
;
Jinsong JIA
1
;
Ting ZHAO
1
;
Jing WANG
1
;
Shenmiao YANG
1
;
Lei WEN
1
;
Jin LU
1
;
Xiaojun HUANG
1
Author Information
- Publication Type:Journal Article
- MeSH: Acute Disease; Chromosomes, Human, Pair 11; Female; Flow Cytometry; Gene Rearrangement; Humans; Leukemia; diagnosis; genetics; Male; Myeloid-Lymphoid Leukemia Protein; genetics; Phenotype; Polymerase Chain Reaction; Prognosis; Proto-Oncogenes; Recurrence; Remission Induction; Retrospective Studies; Young Adult
- From: Chinese Journal of Hematology 2015;36(10):840-843
- CountryChina
- Language:Chinese
-
Abstract:
OBJECTIVETo analyze the clinical features and prognosis of acute leukemia patients with the mixed lineage leukemia(MLL)gene rearrangements AF10 positive.
METHODS6 cases with MLL-AF10 positive were analyzed retrospectively, related literatures were reviewed to clarify MLL-AF10 patients'clinical features and prognosis.
RESULTSThe median age of 6 cases was 19.5 years old, 5 patients with fever onset, the onset white blood cells of 4 patients were less than 10×10⁹/L. 5 cases were as M₅ and 1 case M₄ according to FAB classification, the level of fusion gene(RQ-PCR)was 0.23%-22.60% when diagnosed, 4 cases had concomitant WT1 gene mutation, flow cytometry disclosed myeloid phenotype. Of 5 evaluated patients achieved the first complete remission after conventional chemotherapy, 2 cases of complex karyotype died, one case died of sepsis in induction, another died from failing of transplantation. 4 out of 5 transplant recipients gained long term survival.
CONCLUSIONThe MLL-AF10 positive patients were mostly young men, the majority FAB classification was M5 or M4, often onset with fever, low white blood cells and low level of fusion gene, usually associated with WT1 mutation. Conventional chemotherapy produced a high response rate, but easy to relapse, while the complex karyotype had a poor prognosis, allo-HSCT may have the potential to improve the prognosis of MLL-AF10 positive patients.