Acute myeloid leukemia (AML) is a rare type of childhood acute leukemia, which has a worse prognosis than childhood acute lymphoblastic leukemia. Over the past decade, significant progress has been made in the treatment of childhood AML and the 5-year event-free survival rate may be as high as 70% in developed countries. This survival improvement is largely attributable to risk-stratified treatments, therapies tailored to individual patients based on the biological characteristics of the disease, and continuously improving supportive care. An accurate diagnosis is the prerequisite for risk stratification, prognostic evaluation and therapeutic decision making. How to reduce early mortality and thus improve overall survival, how to implement appropriate supportive treatment to reduce treatment-associated complications, and how to reduce treatment-related mortality are the key to the improvement of therapies for childhood acute myeloid leukemia.
Child ; Humans ; Leukemia, Myeloid, Acute ; diagnosis ; drug therapy ; genetics ; mortality ; Leukemia, Promyelocytic, Acute ; drug therapy ; genetics

Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Bone Marrow Transplantation ; Cytarabine ; administration & dosage ; Humans ; Leukemia, Myeloid, Acute ; diagnosis ; genetics ; therapy ; Prognosis

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.
Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Female ; Hematopoietic Stem Cell Transplantation ; Histone-Lysine N-Methyltransferase ; Humans ; Infant ; Leukemia ; classification ; diagnosis ; genetics ; therapy ; Leukemia, Myeloid, Acute ; diagnosis ; genetics ; therapy ; Male ; Middle Aged ; Myeloid-Lymphoid Leukemia Protein ; genetics ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; diagnosis ; genetics ; therapy ; Prognosis ; Survival Rate ; Young Adult

The study was aimed to investigate the clinical characteristics of acute myeloid leukemia (AML) with CBFB-MYH11 gene. The clinical data of 12 cases were analyzed retrospectively, including age, clinical characteristics, immunophenotype, treatment protocols and efficacy as well as the prognosis. The results indicated that 12 patients with CBFB-MYH11 were detected in 293 AML patients. The median age of the 12 patients was 32.5 (21 - 57) years old. According to French-American-British (FAB) classification, 66.7% (8/12) patients was diagnosed as M4Eo and 33.3% patients was diagnosed as M4. At new diagnosis, the median WBC count was 19.8×10(9)/L (2.46 - 164.30×10(9)/L). The WBC count > 100×10(9) was found in 16.7% patients (2/12). The complete remission (CR) rate after 1 and 2 cycles of induction chemotherapy were 83.3% and 16.7% respectively, so the total CR rate was 100%. Estimated 5-year relapse-free survival (RFS) and overall survival (OS) were 80% and 83%, respectively. It is concluded that patients with CBFB-MYH11 are usually M4Eo and M4. Patients with this fusion gene are often associated with high frequency of CD33, CD34, CD117, HLA-DR, CD15, CD64 and CD14 expression. Patients with CBFB-MYH11 have a tendency of higher CR rate, longer RFS and OS.
Adult ; Female ; Humans ; Immunophenotyping ; Induction Chemotherapy ; Leukemia, Myeloid, Acute ; diagnosis ; drug therapy ; genetics ; Male ; Middle Aged ; Oncogene Proteins, Fusion ; genetics ; metabolism ; Prognosis ; Retrospective Studies ; Young Adult

This study was aimed to explore the immunophenotyping characteristics of acute myeloid leukemia (AML) and their correlation with the curative efficacy. The bone marrow or blood samples were collected from 516 patients with newly diagnosed AML, and their immunophenotypes were analyzed by flow cytometry. The results showed that (1) In 516 cases, the ratios of myeloid antigen expression were higher, as follows: MPO 95.0%, CD33 93.0%, CD13 88.8%, CD117 69.4%; and the expressions of CD14, CD15, CD64 and CD71 were lower, meanwhile 145 cases were accompanied with lymphocyte antigen expression, the ratios were as follows: CD7 21.5%, CD19 6.0%, CD2 0.78%, CD10 0.58% and CD20 0.58%; the positive expression rate of CD71 in M6 was 100%, and that of CD64 in M5 was the highest (30.2%); the overall positive rate of CD34 was 57.8%. (2) After first chemotherapy, the complete remission (CR) rate was 64.7%, CR rate of CD34(+) patients was lower than that of CD34(-) in M3 group (P = 0.019). The CR rate of CD34(+) patients was significantly lower than that of CD34(-) in non-M3 group (P = 0.002). The CR rate of CD19(+) patients was higher than CD19(-) (P = 0.028); the CR rate of CD7(+) patients was significantly lower than that of CD7(-) (P = 0.002); the CR rate of CD71(+) patients was lower than that of CD71(-) (P = 0.013); the CR rate of MPO(+) patients was higher than that of MPO(-) (P = 0.015). Between the CR rate of CD11b, CD13, CD33-positive and-negative group, the difference was not statistically significant (P > 0.05). It is concluded that the phenotype is a prerequisite for the diagnosis of AML, and can help to guide the clinical typing, selection of treatment protocols and evaluation of prognosis.
Adolescent ; Adult ; Aged ; Antigens, CD ; genetics ; Female ; Flow Cytometry ; Humans ; Immunophenotyping ; Leukemia, Myeloid, Acute ; diagnosis ; genetics ; therapy ; Male ; Middle Aged ; Prognosis ; Treatment Outcome ; Young Adult

OBJECTIVEAcute myeloblastic leukemia (AML) accounts for 15 to 25 percent of childhood acute leukemias. Cytogenetic information is important for diagnosis, classification and prognosis of AML. Our aim was to analyze the relationship between karyotypic characteristics and prognosis of childhood AML.

METHODAccording to karyotypic characteristics, 128 newly diagnosed children AML were separated into 4 subgroups: patients with t(15;17) (group APL), patients with t(8;21)/inv(16) (group A), patients with -7/t(9;22)/complex karyotypes (group C) and the others (group B). Prognoses of these patients were analyzed.

RESULTSThe ages ranged from 1 to 16 years with the mean age of 7 years. 85 boys and 43 girls were included in this study. The 4-year event-free survival (EFS) and overall survival (OS) rates were (55.9 ± 4.7)% and (69.3% ± 4.5)%, respectively. The 4-year EFS and OS of non-M(3)-AML patients were (49.9 ± 5.2)% and (57.1 ± 6.0)%, respectively. The probabilities of 4-year EFS of the four subgroups were (72.2 ± 1.1)%, (66.3 ± 7.7)%, (38.5 ± 9.1)% and (20.1 ± 12.3)%, respectively (P = 0.000). The probabilities of 4-year OS were (92.6 ± 5.1)%, (69.4 ± 7.9)%, (55.6 ± 8.6)% and (30.0 ± 12.3)%, respectively (P = 0.000).

CONCLUSIONCytogenetic aberrations seen in pediatric AML had a significant impact on prognosis.

Adolescent ; Child ; Child, Preschool ; Chromosome Aberrations ; Chromosome Disorders ; genetics ; Female ; Humans ; Infant ; Karyotyping ; Leukemia, Myeloid, Acute ; diagnosis ; drug therapy ; genetics ; Male ; Prognosis ; Treatment Outcome

OBJECTIVETo explore clinical and experimental features of 28 cases of childhood acute myeloid leukemia (AML) with 11q23/MLL gene rearrangements.

METHODSKaryotypes of 234 cases of de novo childhood AML were analyzed using short-term culture of bone marrow cells and R-banding. The fusion transcripts involving MLL gene and partial tandem duplication of MLL (MLL-PTD) were detected by multiple reverse transcription polymerase chain reaction (RT-PCR) assay. Two cases with 11q23 translocation by karyotypic analysis but with negative result of multiple RT-PCR were studied with MLL-dual-color fluorescence in situ hybridization (D-FISH).

RESULTSR-banding karyotypic analysis has revealed 20 cases with 11q23 translocation (14 cases with M5, 4 cases with M4, 2 cases with M2), including 12 cases with t(9;11)(p22;q23), 3 cases with t(1;11)(q21;q23), 2 cases with t(6;11)(q27;q23), 1 case with t(11;19)(q23;p13), 1 with t(5;11)(q31;q23), and 1 with t(X;11)(q24;q23). Eighteen cases with 11q23 translocation having fusion transcripts involving MLL genes were confirmed with multiple RT-PCR; 2 cases showed negative results, but they were confirmed to have MLL rearrangements by D-FISH. MLL-PTD was also detected in 8 cases (4 cases M5, 2 cases M4, M2 and M6, one case each) from the other childhood AML cases. The total incidence of 11q23/MLL gene rearrangements was 11.97% (28/234), and most of patients(85.7%, 24/28) were M4/M5. The complete remission (CR) rate after treatment for the 28 cases with MLL rearrangements was 53.8%, the difference was significant by statistics (P< 0.05) compared with 90.5% for the control group (M4/M5 childhood AML with other karyotypic abnormalities or normal karyotype). Of them, 2 cases receiving intensive chemotherapy survived for 81 and 66 months, respectively, 4 cases receiving allogeneic stem cell transplantation survived for 21, 20, 16 and 11 months, respectively, and are still alive with CR. The medium survival (MS) time for 28 cases with 11q23/MLL rearrangements was 11 months, whereas the MS for control group was 15 months. The difference was not statistically significant(P> 0.05).

CONCLUSIONThe 11q23/MLL rearrangements is highly correlated with the occurrence of monocytic leukemia (M4 and M5). The 11q23 translocation and MLL-PTD are mutually exclusive, though both are indicative of poor prognosis. Intensive chemotherapy and allogeneic stem cell transplantation may ameliorate the clinical outcome. Multiple RT-PCR combined with karyotypic analysis and D-FISH are useful for screening the 11q23/MLL rearrangements in childhood AML.

Adolescent ; Child ; Child, Preschool ; Chromosomes, Human, Pair 11 ; Female ; Humans ; Immunophenotyping ; In Situ Hybridization, Fluorescence ; Infant ; Karyotyping ; Leukemia, Myeloid, Acute ; diagnosis ; drug therapy ; genetics ; mortality ; Male ; Myeloid-Lymphoid Leukemia Protein ; genetics ; Remission Induction ; Translocation, Genetic ; Treatment Outcome

No abstract available.
Antineoplastic Agents/*adverse effects/*therapeutic use ; Bone Marrow Cells/metabolism ; Humans ; Karyotyping ; Leukemia, Myeloid, Acute/*chemically induced/*diagnosis/genetics ; Leukemia, Promyelocytic, Acute/*drug therapy ; Male ; Middle Aged ; Oncogene Proteins, Fusion/genetics ; Remission Induction ; Tretinoin/therapeutic use

OBJECTIVETo report two de novo acute myeloid leukemia (AML) patients with t(11;22)(q23;q11.2) and summarize the clinical and biological characteristics.

METHODSBone marrow cells morphology, immunophenotype, chromosome karyotype, fluorescence in situ hybridization (FISH), PCR and gene sequencing were performed. Clinical manifestation and routine laboratory tests were analyzed.

RESULTSThe patients were diagnosed as AML-M₂ and AML-M₅ by morphology and immunophenotype results. Both patients carried t(11;22)(q23; q11.2) and one of them carried an additional chromosome abnormality. MLL-SEPTIN5 fusion transcript was identified in two patients by RT-PCR and sequencing. The two patients got hematologic complete remission after induction chemotherapy with daunorubicin, homoharringtonine, and cytarabine (DHA) or daunorubicin and cytarabine (DA). One of them relapsed and died during consolidation therapy with intermediate-dose cytarabine.

CONCLUSIONLeukemia with t(11;22)(q23;q11.2) chromosome translocation met the clinical and laboratory manifestations of AML. The MLL-SEPTIN5 fusion transcript was the distinctively biological etiology. Patients with t(11;22)(q23;q11.2) were vulnerable to relapse after conventional chemotherapy and had poor prognosis. Allogeneic hematopoietic stem cell transplantation should be recommended as early as possible.

Adult ; Chromosome Aberrations ; Chromosomes, Human, Pair 11 ; Chromosomes, Human, Pair 22 ; Female ; Humans ; Karyotyping ; Leukemia, Myeloid, Acute ; diagnosis ; drug therapy ; genetics ; Male ; Prognosis ; Translocation, Genetic

Tsukamurella pulmonis is an aerobic actinomycete. We report a catheter-related bacteremia of T. pulmonis. A 39 yr-old male with ALL was hospitalized to receive bone marrow transplantation (BMT). Although the patient developed a high fever at the 7th hospital day (HD), it subsided with vancomycin treatment, and he received BMT at 9th HD. Fever resurged at 16th HD despite sustained treatment with vancomycin, meropenem, and amphotericin B, but subsided with removal of Hickman catheter (HC) at 19th HD. Three sets of blood cultures comprising one from the HC and two from venipunctures were taken at 7th, 16th, and 19th HD, and the distal tip of the HC was also cultured. The aerobic vials of all 3 HC-withdrawn blood cultures and one peripheral blood culture taken at 19HD and the HC tip culture grew long, straight, thin gram-positive rods that were positive on modified Kinyoun stain. This organism showed tiny, rough, grey colonies after 3-day incubation and grew to large flat colonies when incubation was extended. It was catalase-positive, urease-positive, and alkaline-slant/alkaline-deep on triple sugar iron agar, and hydrolyzed hypoxanthine. The sequence of 1,296 base pairs of 16S rRNA of this organism showed a 100.0% homology with the published sequence of T. pulmonis DSM 44142T. To our knowledge, this is the first report of T. pulmonis bacteremia in Korea.
Actinomycetales/classification/genetics/isolation & purification ; Actinomycetales Infections/diagnosis/*microbiology/therapy ; Adult ; Bacteremia/*diagnosis/microbiology/therapy ; Bone Marrow Transplantation ; Catheter-Related Infections/*microbiology ; Humans ; Leukemia, Myeloid, Acute/therapy ; Male ; Phylogeny ; RNA, Ribosomal, 16S/genetics