title: [Clinical and molecular biologic characteristics of 36 cases of leukemia with 11q23/mll].
abstract: This study was aimed to analyze the clinical and cytogenetic characteristics of acute leukemia with 11q23/mll rearrangement and explore the reasonable therapeutic principles. Characteristics in general situation, morphology, immunology, molecular biology, cytogenetics, treatment and overall survival of 36 cases of acute leukemias with mll gene rearrangement were studied and analyzed. The results showed that 36 cases with mll gene rearrangement were found positive (7.2%) in 494 patients with acute leukemia. Among the 36 cases of mll rearrangement positive, 32 cases were diagnosed as acute myeloid leukemia (AML) with myeloid antigen expression, of which 5 cases expressed lymphoblastic differentiation antigen; 4 cases were classified as B-lineage acute lymphoblastic leukemia (ALL), of which non-lineage myeloid expression pattern were found in 3 cases. In 29 out of 36 cases (80%) the clonal chromosomal aberration were detected, of which chromosome 11 aberration were observed in 22 cases. All patients received chemotherapy with a total response rate of 47.2%. Of the responded patients, 10 cases relapsed within 6 months, with a recurrence rate of 40%; 9 cases received hematopoietic stem cell transplantation (HSCT), 7 cases of which survived after transplantation. The median survival time of 36 cases was 16 months (range 2 - 46) and their 2-year overall survival rate was 41.4%. The 2-year overall survival rate of 9 patients who received HSCT was 87.5%. It is concluded that acute leukemia patients with mll gene rearrangement show poor response to chemotherapy, high recurrence rate and poor prognosis. Hematopoietic stem cell transplantation may be a reasonable treatment principle to improve these patients' survival situation.
authors.affiliation: Beijing Daopei Hospital, Beijing 100049, China.
authors.forename: Jie
authors.initials: J
authors.lastname: Zhao
authors.forename: Yu-Ming
authors.initials: YM
authors.lastname: Yin
authors.forename: Yan-Li
authors.initials: YL
authors.lastname: Zhao
authors.forename: Yuan
authors.initials: Y
authors.lastname: Sun
authors.forename: Jing-Bo
authors.initials: JB
authors.lastname: Wang
authors.forename: Jing
authors.initials: J
authors.lastname: Zhong
authors.forename: Xian
authors.initials: X
authors.lastname: Zhang
authors.forename: Xin-Hong
authors.initials: XH
authors.lastname: Fei
authors.forename: Fu-Xiang
authors.initials: FX
authors.lastname: Shan
authors.forename: Hong-Xing
authors.initials: HX
authors.lastname: Liu
authors.forename: Tong
authors.initials: T
authors.lastname: Wang
authors.forename: Hui
authors.initials: H
authors.lastname: Wang
authors.forename: Chun-Rong
authors.initials: CR
authors.lastname: Tong
authors.forename: Tong
authors.initials: T
authors.lastname: Wu
authors.forename: Dao-Pei
authors.initials: DP
authors.lastname: Lu
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D011495:Histone-Lysine N-Methyltransferase; D006801:Humans; D007223:Infant; D007938:Leukemia / Q000145:classification / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D015470:Leukemia, Myeloid, Acute / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D008297:Male; D008875:Middle Aged; D051788:Myeloid-Lymphoid Leukemia Protein / Q000235:genetics*; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D011379:Prognosis; D015996:Survival Rate; D055815:Young Adult
chemical_list: C497422:KMT2A protein, human; D051788:Myeloid-Lymphoid Leukemia Protein; D011495:Histone-Lysine N-Methyltransferase