Progress of immunotherapy in pediatric lymphocyte malignancies / 国际儿科学杂志

In the last half-century, the multi-cooperation of conventional therapies including chemotherapy, radiotherapy and bone marrow hematopoietic stem cell transplantation has saved a lot of children with lymphocyte malignancies, but the outcomes of relapsed and/or refractory diseases remain dismal.Thus, it is in urgent need of novel approaches to improve clinical efficiency.After decades of rapid development, immunotherapy has achieved remarkable results from the treatment of pediatric lymphocyte malignancies, especially chimeric antigen receptors T(CAR-T), bi-specific T cell engager(BiTE), and immune checkpoint blockader(ICB). However, a part of patients is still resistant to immunotherapy.The review will focus on CAR-T, BiTE and ICB to elucidate the pre-clinical studies and clinical trials that have been intensively conducted in pediatric leukemia and lymphoma, as well as the brief introduction of their limitations and probably optimal strategies.

Oral health management in children with acute lymphoblastic leukemia / 口腔疾病防治

@#Acute lymphoblastic leukemia is the most common type of leukemia in children. In recent years, the treatment and prognosis of acute lymphoblastic leukemia in children have improved significantly. However, acute lymphoblastic leukemia itself and treatment measures can lead to a variety of adverse oral complications and affect further treatment. These complications have a significant effect on patients and affect further treatment. To improve the quality of life of children, this article reviews the oral health status and oral health management of children with acute lymphoblastic leukemia for clinical reference. The literature review shows that the oral health management measures for children with acute lymphoblastic leukemia mainly include oral health education, active prevention of oral diseases, timely oral treatment before the start of antitumor treatment, elimination of potential sources of infection after blood examination and risk assessment, treatment of oral mucosal problems during therapy, oral emergency during antitumor therapy should be carried out under the guidance of hematologists and regular oral inspection and oral care. However, current studies have found that there is still a lack of effective prevention and treatment measures for oral mucositis. As a common oral disease in children with acute lymphoblastic leukemia, the prevention and treatment of oral mucositis remain to be further studied.

Advance in treatment of pediatric leukemia through modulating signaling pathways induced by traditional Chinese medicine / 中国中药杂志

Leukemia is the most common type of cancer in childhood, which is also one of the malignant tumors that can be cured by chemotherapy. However, infection and the side effects of chemotherapy drugs are the causes of death in children with leukemia. Chemotherapy combined with traditional Chinese medicine is used to treatment of pediatric leukemia in clinic. Although traditional Chinese medicine has extensive clinical experience in the treatment of leukemia, the mechanism is not clear. The targeted drugs are attractive for the advantages of the high selectivity and little side effects. Targeted therapies in pediatric leukemia are targeting BCR/ABL, TARA and FLT3 proteins, which activation results in the downstream activation of multiple signaling pathways, including the PI3K/AKT, JNK/STAT, Ras/ERK pathways. In recent years, the target site or the signaling pathways are clear for the traditional Chinese medicine in the treatment of leukemia. In this paper, we review recent studies, summary the traditional Chinese medicine prescriptions and effective ingredients which can regulate the signaling pathways. We view to provide a basis for the treatment of childhood leukemia with traditional Chinese medicine combined with chemotherapy drugs or targeted drugs.

Prevalence and Clinical Implication of Partial Tandem Duplication of the Mixed Lineage Leukemia Gene in Pediatric Acute Leukemia / 임상소아혈액종양

BACKGROUND: The mixed lineage leukemia (MLL) gene may induce hematopoiesis and leukemia. Partial tandem duplication of MLL (MLL-PTD) is associated with poor prognosis in acute myeloid leukemia (AML); however, the significance of MLL-PTD in acute lymphoblastic leukemia (ALL) has not been thoroughly studied. We evaluated the incidence, relationship with other cytogenetic abnormalities, and the prognostic role of MLL-PTD in ALL.METHODS: We reviewed medical records from pediatric patients diagnosed with ALL in Severance Hospital, Yonsei University Health System, South Korea from 2002 to 2008. MLL-PTD was detected by nested reverse transcriptase polymerase chain reaction.RESULTS: In ALL patients, 50.0% (42/84) were positive for MLL-PTD. There was no significant difference in the 10-year overall survival (10Y OS) and event-free survival (EFS) between MLL-PTD-positive (+) and MLL-PTD-negative (-) groups (69.4% vs. 76.2%, P=0.609, and 62.6% vs. 66.7%, P=0.818, respectively). The combination of high level of lactate dehydrogenase (>1,100 IU/L) and MLL-PTD(+) [MLL-PTD(+)/High LDH] was a statistically significant negative prognostic factor for 10Y OS and EFS (P=0.0226 and P=0.0230, respectively). In multivariate analysis, National Cancer Institute risk stratification and very high risk features were independent significant prognostic factors but MLL-PTD (+)/High LDH was not.CONCLUSION: MLL-PTD was observed frequently in pediatric ALL patients. MLL-PTD was not an independent prognostic factor. MLL-PTD (+)/High LDH should be evaluated further for its prognostic potential in ALL.

Prevalence and Clinical Implication of Partial Tandem Duplication of the Mixed Lineage Leukemia Gene in Pediatric Acute Leukemia / 임상소아혈액종양

BACKGROUND: The mixed lineage leukemia (MLL) gene may induce hematopoiesis and leukemia. Partial tandem duplication of MLL (MLL-PTD) is associated with poor prognosis in acute myeloid leukemia (AML); however, the significance of MLL-PTD in acute lymphoblastic leukemia (ALL) has not been thoroughly studied. We evaluated the incidence, relationship with other cytogenetic abnormalities, and the prognostic role of MLL-PTD in ALL. METHODS: We reviewed medical records from pediatric patients diagnosed with ALL in Severance Hospital, Yonsei University Health System, South Korea from 2002 to 2008. MLL-PTD was detected by nested reverse transcriptase polymerase chain reaction. RESULTS: In ALL patients, 50.0% (42/84) were positive for MLL-PTD. There was no significant difference in the 10-year overall survival (10Y OS) and event-free survival (EFS) between MLL-PTD-positive (+) and MLL-PTD-negative (-) groups (69.4% vs. 76.2%, P=0.609, and 62.6% vs. 66.7%, P=0.818, respectively). The combination of high level of lactate dehydrogenase (>1,100 IU/L) and MLL-PTD(+) [MLL-PTD(+)/High LDH] was a statistically significant negative prognostic factor for 10Y OS and EFS (P=0.0226 and P=0.0230, respectively). In multivariate analysis, National Cancer Institute risk stratification and very high risk features were independent significant prognostic factors but MLL-PTD (+)/High LDH was not. CONCLUSION: MLL-PTD was observed frequently in pediatric ALL patients. MLL-PTD was not an independent prognostic factor. MLL-PTD (+)/High LDH should be evaluated further for its prognostic potential in ALL.

A Case of Acute Megakaryoblastic Leukemia Diagnosed by t(1;22)(p13;q13) / 대한진단검사의학회지

A four-year-old female initially presented with fever, cough, headache and bone pain. On admission, a complete blood cell count revealed anemia (Hb 8.4 g/dL, WBC 4, 630/microL, platelets 132, 000/microL) and a few blasts were observed in a peripheral blood smear. A bone marrow study revealed inadequate aspirate due to dry tap and extensive fibrosis on the biopsy section. Cytogenetic analysis showed a karyotype with 48, XX, t(1;22)(p13;q13), +der(1) t(1;22), +2. Considering the specificity of cytogenetic results and extensive myelofibrosis, acute megakaryoblastic leukemia was diagnosed. Acute megakaryoblastic leukemia with t(1;22)(p13;q13) is known to be a relatively clear-cut cytogeneticomorphological defined syndrome. Herein, we report a first case of acute megakaryoblastic leukemia with t(1;22)(p13;q13) in Korea.