Childhood acute lymphoblastic leukemia (ALL) accounts for about 75% of childhood leukemia cases, and B-lineage acute lymphoblastic leukemia (B-ALL) accounts for more than 80% of childhood ALL cases. Over the past half century, new molecular biological targets discovered by new techniques have been used in precise stratification of disease prognosis, and there has been a gradual increase in the 5-year overall survival rate of childhood ALL. With the increasing attention to long-term quality of life, the treatment of childhood B-ALL has been constantly optimized from induction therapy to the intensity of maintenance therapy, including the treatment of extramedullary leukemia without radiotherapy, which has been tried with successful results. The realization of optimized treatment also benefits from the development of new techniques associated with immunology and molecular biology and the establishment of standardized clinical cohorts and corresponding biobanks. This article summarizes the relevant research on the implementation of precise stratification and the intensity reduction and optimization treatment of B-ALL in recent years, providing reference for clinicians.
Humans ; Quality of Life ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy* ; Prognosis ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma/drug therapy* ; Acute Disease

OBJECTIVES: Rate of complete remission and long-term survival in adult acute lymphoblastic leukemia group has not been as satisfactory as that in childhood ALL. Recently introduction of induction chemotherapy of more intensive combination and various trials of postremission therapy are making improved results better looked forward to. And subtypes of ALL according to the degree of differentiation into T and B cells are identified by using immunologic markers hopefully to work out proper treatment for each subtype. METHODS: We analited results of treatment and differences of complete remission rate, remission duration and overall survival as to various immunologic markers and clinicopathologic characteristics in 33adult ALL patients. RESULTS: Eighty five percents of the 27cases that had VPDL chemotherapy achieved complete remission and both overall median survival and mediom duration of remission were 52weeks. No definite prognostic factors were detected influencing complete remission rate, remission duration and overall survival except that patients with serum albumin level higher than 4.0mg/dL showed highter complete remission rate. Although mature B-ALL showed the shortest overall median survival, degree of differenciation of B-cell and other immunologic markers did not influence on complete remission rate, remission duration or overall survival. CONCLUSION: Further studies are needed to delire the prognostic factors in adult ALL.
Adult ; B-Lymphocytes ; Biomarkers ; Drug Therapy ; Humans ; Induction Chemotherapy ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Serum Albumin

B-Lymphocytes ; Child ; Humans ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; drug therapy ; immunology ; Precursor Cells, B-Lymphoid ; pathology

Humans ; Treatment Outcome ; Antibodies, Bispecific/adverse effects* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy* ; Antineoplastic Agents/adverse effects* ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma

We experienced a 22-year old patient with a documented history of minimal change nephrotic syndrome (MCNS), and a diagnosis of acute lymphoblastic leukemia (ALL) was then made for this patient. The patient received standard daily steroid therapy for the treatment of nephrotic syndrome. Cyclosporin A was administered because there was no clinical improvement with steroid therapy. Six years after the diagnosis of nephrotic syndrome, the patient was diagnosed with ALL. After chemotherapy for ALL, the patient was in complete remission and he showed clinical improvement of nephrotic syndrome. The hematological malignancies associated with nephrotic syndrome are mainly lymphoma and chronic lymphocytic leukemia. ALL has rarely been described in combination with nephrotic syndrome. Although the exact mechanism for development of ALL after nephrotic syndrome is unknown, at least two possibilities exist. First, the incidence of leukemia may be increased after immunosuppressive therapy, which may include cyclosporin A. Second, the underlying defect in T-lymphocyte function could account for both nephrotic syndrome and ALL. The possible mechanisms for such a relationship are discussed here along with a review of the relevant literature.
Cyclosporine ; Diagnosis ; Drug Therapy ; Hematologic Neoplasms ; Humans ; Incidence ; Leukemia ; Leukemia, Lymphocytic, Chronic, B-Cell ; Lymphoma ; Nephrosis, Lipoid* ; Nephrotic Syndrome ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; T-Lymphocytes ; Young Adult

BACKGROUND: Lymphocytes seen during the chemotherapy of childhood ALL are not fully understood regarding their clinical significance. The lymphoid aggregates found during the complete remission period are more confusing. We investigated the characteristics of lymphoid aggregates and the clinial course of children with these in the marrow during the chemotherapy of childhood ALL. This is the first study about this subject. METHODS: From January 1996 to April 1998, 210 bone marrow specimens were diagnosed as complete remission status of ALL and among them, ten patients (4.8%) showed lymphoid aggreagates on the marrow clot sections at the time of complete remission. We reviewed bone marrow specimens, performed immunohistochemical stains for CD3, CD 10 and CD79a and investigated the clinical course. RESULTS: The ten cases were composed of nine ALL, L1 and one ALL, L2. All of them were treated under guidance of the CCG (children's cancer group) protocol. Fourteen lymphoid aggregates from ten cases were found. They showed mean number of 1.4 per clot section, mean diameter of 132 micrometer, regular (36%) or irregular (64%) margin and composition of mature lymphocytes (21%), immature lymphocytes (29%) or mixed pattern (50%). The mean interval between the diagnosis and the emergence of lymphoid agregates was 29 months (2~55 months). One patient in the course of consolidation chemotherapy expired due to upper gastrointestinal bleeding and other nine cases are still in the continuous complete remission state. The lymphoid cells consisting of lymphoid aggregates showed positive reaction only for CD79a and negative reactions for CD3 and CD10. CONCLUSION: Lymphpoid aggregates found at the time of complete remission are collections of regenerating B-lymphocytes and they are not residual leukemic blasts, and show no effect on the complete remission state.
B-Lymphocytes ; Bone Marrow* ; Child ; Coloring Agents ; Consolidation Chemotherapy ; Diagnosis ; Drug Therapy ; Hemorrhage ; Humans ; Lymphocytes ; Precursor Cell Lymphoblastic Leukemia-Lymphoma

Precursor B-cell lymphoblastic lymphoma (B-LBL) is an uncommon subtype of Non-Hodgkin lymphoma (NHL), accounting for only 0.3% of NHL in adults and less than 10% of all LBL cases. Unlike T-cell LBL, it usually presents with extranodal involvement while sparing the bone marrow (BM). Among the 27 patients with LBL treated in the Asan Medical Center between January 2007 and March 2012, 3 had B-LBL. All had a good performance status and low International Prognostic Index. However, unlike most previously reported cases, the patients had lymphoma in their bone marrow and extranodal sites such as bone and lung. After intensive combination chemotherapy, one patient achieved a complete response and the other 2 patients, a partial response. Our experience suggests that multiple extranodal sites may be involved in B-LBL and BM involvement may not be as infrequent as previously thought. Furthermore, intensive chemotherapy seems to be effective.
Adult ; Bone Marrow ; Chungcheongnam-do ; Drug Therapy ; Drug Therapy, Combination ; Humans ; Lung ; Lymphoma ; Lymphoma, Non-Hodgkin ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma* ; T-Lymphocytes

BACKGROUND: Precursor T-cell acute lymphoblastic leukemia (T-ALL) has worse prognosis than B-cell ALL. We aimed to evaluate prognostic variables in pediatric T-ALL. METHODS: Medical records of 36 T-ALL patients (27 males and 9 females; median age at diagnosis, 10.6 years) diagnosed and treated at Asan Medical Center from 2001 to 2017 were reviewed. Six patients (16.7%) had early T-cell precursor ALL (ETP-ALL). Most patients received the Children's Cancer Group-1882 (CCG1882) or Korean multicenter high risk ALL (ALL0601) protocols and prophylactic cranial irradiation. Clinical features at presentation, response to therapy, and treatment outcomes were analyzed. RESULTS: The six patients with ETP-ALL and 17 of 30 with non-ETP-ALL received CCG1882 or ALL0601 chemotherapy. Three patients, including two with ETP-ALL, did not achieve complete remission after induction. Rapid early response during induction was achieved by 26 patients. Five year overall survival (OS) and event free survival (EFS) rates were 71.4% and 70.2%, respectively. ETP-ALL and slow early response during induction were significant adverse prognostic factors, while hyperleukocytosis at diagnosis was not. CCG1882/ALL0601 chemotherapy resulted in superior survival (OS: 78.9%, EFS: 73.3%) compared with CCG1901 chemotherapy (OS: 64.3%, EFS: 64.3%), and patients undergoing prophylactic cranial irradiation had superior EFS to non-radiated patients. CONCLUSION: A high risk ALL protocol with intensified post-remission therapy, including prophylactic cranial irradiation, conferred T-ALL survival outcomes comparable with those of Western studies. Further treatment intensification should be considered for patients with ETP-ALL and slow induction responders. Additionally, CNS-directed treatment intensification, without prophylactic cranial irradiation, is needed.
B-Lymphocytes ; Chungcheongnam-do ; Cranial Irradiation ; Diagnosis ; Disease-Free Survival ; Drug Therapy ; Female ; Humans ; Male ; Medical Records ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Precursor Cells, T-Lymphoid ; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma ; Prognosis ; T-Lymphocytes*

OBJECT: Pre-ALL is a very rare preteukemic state, which percedes acute lymphocytic leukemia, while MDS(pre-ANLL), usually the well-known type of preleukemic state, precedes acute non-lymphocytic leukemia. Initially it shows transient pancytopenia without any evidence of leukemia in bone marrow findings, followed by acute lymphocytic leukemia after recovery from pancytopenia of a short period within weeks or months. We report a case with pre-ALL in childhood. CASE: A 15-month-old male baby was admitted with the complaints of fever and cough for 5 days and pallor for 2 weeks prior to admission. On admission, CBC showed pancytopenia without any evidence of leukemia, which was recovered spontaneously in a short period, and then was followed by acute lymphocytic leukemia of CALLA negative, early pre-B cell type. During antileukemic chemotherapy, he had suffered from severe bacterial infections and was finally died of sepsis 8 months after first admission. CONCLUSION: We report a case of pre-ALL in childhood, which was preceded by CALLA negative, early pre-B cell ALL, with a review of the literatures, briefly.
Bacterial Infections ; Bone Marrow ; Cough ; Drug Therapy ; Fever ; Humans ; Infant ; Leukemia ; Male ; Pallor ; Pancytopenia ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Precursor Cells, B-Lymphoid ; Sepsis

OBJECTIVE: To explore the consistency of flow cytometry (FCM) method and polymerase chain reaction (PCR) technique in the detection of minimal residual disease (MRD) at different treatment stages in pediatric patients with TCF3/PBX1⁺ B-cell acute lymphoblastic leukemia (B-ALL) and the correlations between the detection results and prognosis. METHODS: The clinical data of 64 newly diagnosed pediatric patients with TCF3/PBX1⁺ B-ALL admitted to the Department of Pediatrics of Peking University People's Hospital from January 2005 to December 2017 were retrospectively analyzed. FCM and PCR methods were used to monitor the MRD level in bone marrow samples from 64 children during the same period of treatment on d33 and d90 respectively, and the detection results were analyzed. RESULTS: There were 37 males and 27 females in the 64 patients, with a median age of 8 years(range 0.8 to 16 years). The complete remission (CR) rate after the first cycle of induction chemotherapy was 98.4% (62/63), with overall CR rate of 100%. 12 patients experienced recurrence, with a median recurrence time of 16.9 (5.3-46.3) months. The median follow-up time of the 64 patients was 77.2 (1.0-184.8) months , and the 5-year overall survival (OS) rate and event-free survival (EFS) rate were 82.8%±4.7% and 75.0%±5.4%, respectively. On d90, the concordance rate of the MRD results from the two methods was 98.4%, and the related kappa value was 0.792 (P < 0.001), which were significantly higher than those on d33. After induction chemotherapy (d33), the 5-year EFS rate of MRD-FCM^- group (79.3%±5.3%) was significantly better than that of MRD-FCM⁺ group (40.0%±21.9%) (P =0.028), there were no significant differences in the 5-year OS rate and EFS rate between MRD-PCR⁺ group and MRD-PCR^- group, and the 5-year EFS rate of MRD-FCM^-/PCR^- group (85.4%±5.5%) was significantly better than that of MRD-FCM⁺/PCR⁺ group (40.0 %±21.9%) (P =0.026). CONCLUSION In children with TCF3/PBX1⁺ B-ALL, the MRD results detected by FCM and PCR methods show good consistency, especially in consolidation therapy period (d90). The MRD level at the end of induction therapy (d33) is an important factor affecting the long-term prognosis, especially the MRD results detected by FCM method, which is significantly associated with prognosis.
Male ; Female ; Child ; Humans ; Infant ; Child, Preschool ; Adolescent ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy* ; Neoplasm, Residual/diagnosis* ; Clinical Relevance ; Retrospective Studies ; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma ; Prognosis ; Burkitt Lymphoma ; Basic Helix-Loop-Helix Transcription Factors/therapeutic use*