Efficacy of ruxolitinib for HAVCR2 mutation-associated hemophagocytic lymphohistiocytosis and panniculitis manifestations in children.

Frequent germline mutations of HAVCR2, recently identified in subcutaneous panniculitis-like T-cell lymphoma (SPTCL), are associated with an increased risk of hemophagocytic lymphohistiocytosis (HLH). However, SPTCL-HLH represents a challenge because of the difficulties in treatment with poor survival. Its malignant nature, specifically harbouring HAVCR2 mutations, has also been questioned.

Diagnosis and treatment of pediatric anaplastic lymphoma kinase-positive large B-cell lymphoma: A case report.

Background: Anaplastic lymphoma kinase-positive (ALK+) large B-cell lymphoma (LBCL) is a rare type of lymphoma with high invasiveness and rapid progression. It occurs in all age groups, but is extremely rare in children. The lesions mainly involve the lymph nodes and may present with extra-nodal involvement.

Treatment outcome in children with central nervous system-positive Burkitt lymphoma using only intrathecal and systemic chemotherapy combined with rituximab.

Background: With current chemotherapy treatment, >90% of survival has been obtained for Burkitt lymphoma (BL). In this study, the demographic characteristics and treatment outcomes are presented for 78 children in China with central nervous system-positive (CNS+) BL.

Methods: This retrospective study consecutively enrolled 78 CNS+ BL patients in Beijing Children's Hospital (BCH) from 2007 to 2019 who received the BCH B-cell non-Hodgkin's lymphoma regimen (modified by French-American-British mature lymphoma B-cell 96 [FAB/LMB96] C1 arm ± rituximab).

Central nervous system relapse in a pediatric anaplastic large cell lymphoma patient with CLTC/ALK translocation treated with alectinib: A case report.

Background: The aberrant expression of the anaplastic lymphoma kinase () gene in ALK-positive (ALK+) anaplastic large cell lymphoma (ALCL) is usually due to t(2;5)/NPM-ALK. However, rarely, aberrant ALK expression can also result from a rearrangement of the gene with various partner genes. Central nervous system (CNS) metastasis is very rare in ALK+ALCL.

[Prognostic significance of the NPM-ALK fusion gene in bone marrow and peripheral blood for patients with anaplastic large cell lymphoma].

Objective: To investigate the expression of NPM- ALK fusion gene in bone marrow (BM) and peripheral blood (PB) in anaplastic large cell lymphoma (ALCL) patients and its prognostic significance.

Methods: NPM- ALK fusion gene of 21 BM and 15 PB samples from patients with NPM-ALK positive ALCL was detected by RT- PCR, and the relationship between NPM- ALK expression and prognosis and clinical characters was evaluated.

Results: Of the 21 patients, 12 cases were male and 9 case were female with a median age of 9 (range, 2-14) years old.

[Clinical features and therapeutic effect of 38 children with anaplastic large cell lymphoma].

Objective: To analyze the clinical features and prognostic factors of children's anaplastic large cell lymphoma (ALCL), summarize the therapeutic effect and toxicities.

Method: A total of 38 ALCL patients admitted to Beijing Children's Hospital from Jan. 2003 to Apr.

[Clinical features and prognosis of children with lymphoblastic lymphoma].

Objective: To evaluate the clinical characteristics of childhood lymphoblastic lymphoma (LBL) and therapeutic efficacy of BCH-LBL-2003 regimen modified from BFM-90 protocol. The drug-related toxicities and prognostic factors were explored at the same time.

Methods: From Janurary 2003 to December 2009, 112 newly diagnosed LBL patients at the Hematology Center of Beijing Children's Hospital were enrolled in this study.

[Clinical analysis of 18 cases with acute tumor lysis syndrome in children with B-cell lymphoma].

Objective: To investigate risk factors associated with acute tumor lysis syndrome (ATLS) in children with B-cell lymphoma and to explore feasible means for the prophylaxis and treatment.

Method: Data from 18 children with ATLS in B-cell lymphoma were collected to assess their tumor burden at diagnosis and before chemotherapy. Evaluation was performed at the 8th day, 3 month, and the end of chemotherapy and follow up.

[Clinical characteristics of 34 children with Hodgkin lymphoma and efficacy of treatment with chemotherapy plus low dose radiotherapy on involved sites].

Objective: To summarize the clinical features and to evaluate outcomes and to assess therapeutic effects in 34 children and adolescents with Hodgkin lymphoma treated with risk-adapted combination chemotherapy and low-dose, involved-field radiation therapy (IFRT) in China.

Method: From January 2003 to April 2009, 34 hospitalized children with Hodgkin lymphoma were enrolled into the BCH-HL 2003 protocol (revised CCG 5942) in our hospital. Pathological samples were reviewed centrally and classified based on the World Health Organization guidelines.

[Clinical analysis of children with lymphoma complicated with severe pneumonia due to novel influenza A (H1N1) virus infection].

Objective: To study the clinical features and treatment of severe pneumonia due to novel influenza A (H1N1) virus in children with lymphoma during chemotherapy.

Method: The clinical manifestations, radiologic features, reasons of misdiagnosis, experiences in treatment and prognosis of 4 children with lymphoma complicated with pneumonia due to novel influenza A (H1N1) virus during chemotherapy were analyzed retrospectively.

Result: Four children out of the 54 patients with hematologic disorders who were receiving chemotherapy suffered from H1N1 influenza.

[Clinical features of hepatitis-associated aplastic anemia in children].

Objective: To study the clinical features of hepatitis-associated aplastic anemia (HAAA) in children.

Methods: The clinical data of the children with newly diagnosed HAAA from January 2007 to December 2008 were respectively studied, including clinical manifestations, and blood routine, bone marrow examination, viral serology and immune function results as well as treatment and prognosis.

Results: A total of 8 children were confirmed as HAAA, accounting for 4.

[Tolerability of 6-mercaptopurine in children with acute lymphoblastic leukemia].

Objective: 6-Mercaptopurine (6-MP) has been the backbone of maintenance chemotherapy for acute lymphoblastic leukemia (ALL), the response to 6-MP is highly variable, adverse events leading to discontinuation or dose-reduction (children intolerant) of 6-MP occur in many children with ALL. The aim of this study was to investigate the tolerability of 6-MP and to optimize thiopurine use.

Methods: The authors evaluated in a prospective manner the tolerance of 6-MP in ALL children from Oct.

[An analysis of mutations causing Gaucher disease in Chinese population].

Objective: To review and investigate the relationship of genotype and phenotype in Chinese patients with Gaucher disease (GD).

Methods: The samples were first screened for known mutations as reported previously in Chinese population. Long chain PCR and nested PCR were employed to amplify the segments of glucocerebrosidase functional gene in patients with unknown mutant alleles.

[Clinical study of 40 children with Burkitt's and Burkitt-like lymphoma].

Objective: To summarize the histological and clinical characteristics of 40 cases with Burkitt's and Burkitt-like lymphoma in children, to evaluate the effects of treatment with international regimen, and to explore the treatment-related complications and prognostic factors.

Methods: Forty patients with Burkitt's and Burkitt-like lymphoma were registered in Beijing Children Hospital from Feb 2003 to Apr 2006. The diagnosis was confirmed by histology and immunohistochemistry of biopsy, and clinical staging by the examination of imaging, cerebrospinal fluid and bone marrow based on St.

Catalpol protects rat pheochromocytoma cells against oxygen and glucose deprivation-induced injury.

Objectives: Catalpol has been identified to have neuroprotective effect on gerbils subjected to transient global cerebral ischemia. However, the mechanism that catalpol prevents ischemia is still unclear. In the present study, PC12 cells, exposed to oxygen and glucose deprivation (OGD) followed by reperfusion, were used as an in vitro model of ischemia.

Efficacy of catalpol as protectant against oxidative stress and mitochondrial dysfunction on rotenone-induced toxicity in mice brain.

Rotenone, a specific inhibitor of mitochondrial complex I, reproduces many features of Parkinson's disease. The aim of the study was carried out to investigate how rotenone affected the mitochondrial function and antioxidant/oxidant parameters of mouse striatum, and secondly, to evaluate the ameliorating effects of catalpol against rotenone-induced damage. Our results showed that rotenone induced significant changes in mitochondrial function such as complex I activity and mitochondrial membrane potential decreased, and enhanced antioxidant status as glutathione depletion, enzymatic (glutathione peroxidase and superoxide dismutase) disorders, and increased lipid peroxidation.

[A retrospective study on enzyme replacement therapy in patients with Gaucher disease].

Objective: Gaucher disease is the most common lysosomal storage disorder. A deficiency of beta-glucocerebrosidase causes accumulation of the glucocerebroside in macrophages throughout the body. This study summarizes the effects of enzyme replacement therapy (ERT) with Imiglucerase in children with Gaucher disease.

Catalpol protects dopaminergic neurons from LPS-induced neurotoxicity in mesencephalic neuron-glia cultures.

Inflammation plays an important role in the pathogenesis of Parkinson's disease (PD). Microglia, the resident immune cells in the central nervous system, are pivotal in the inflammatory reaction. Activated microglia can induce expression of inducible nitric-oxide synthase (iNOS) and release significant amounts of nitric oxide (NO) and TNF-alpha, which can damage the dopaminergic neurons.

Neuroprotection of catalpol in transient global ischemia in gerbils.

The neuroprotection of catalpol and its mechanism was evaluated in cerebral ischemic model in gerbils. Three groups were designed as sham-operated, ischemia-treated, respectively, with catalpol and saline. Catalpol was injected intraperitoneally immediately after reperfusion and repeatedly at 12, 24, 48 and 72 h with the dose of 5.