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.

Clinical features and treatment outcomes of pediatric Langerhans cell histiocytosis with macrophage activation syndrome-hemophagocytic lymphohistiocytosis.

Background: Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm. A few LCH patients had Macrophage activation syndrome-hemophagocytic lymphohistiocytosis (MAS-HLH), a life-threatening, hyper-inflammatory syndrome. We retrospectively described the clinical-biological characteristics of a series of 28 pediatric LCH patients with MAS-HLH in a single center.

[Effect of Film Mulching, Straw Retention, and Nitrogen Fertilization on the NO and N Emission in a Winter Wheat Field].

In order to explore the characteristics of NO emissions from winter wheat fields in the Loess Plateau under different farming methods and nitrogen levels, the dynamic changes in NO emissions from rain-fed winter wheat fields were quantified using static box-gas chromatography. Winter wheat 'Xiaoyan22' was used as the material, and a two-factor split area design was adopted. The conventional tillage (CT), straw incorporated into soil (SM), and flat film mulching (FM) were assigned as the main plot, and three nitrogen fertilizer rates (no nitrogen fertilization, 20% nitrogen reduction (144 kg·hm), and conventional nitrogen application (180 kg·hm)) were assigned as a split plot.

[Case Report: Three Pediatric Pulmonary Cryptococcosis Patients with Prominent Manifestation of Mediastinal Lymphadenopathy].

In this study, we report on three immunocompetent children with pulmonary cryptococcosis presenting mediastinal lymphadenopathy as the prominent manifestation. All three children were otherwise healthy previously. Two children had a history of exposure to pigeons and poultry.

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).

Extraskeletal Ewing's Sarcoma with CD7 Positivity and T-cell Receptor/Immunoglobulin Rearrangement Masquerading as T-lymphoblastic Lymphoma.

Extraskeletal Ewing's Sarcoma (EES) may harbor more than one tumor-specific genetic abnormality, leading to diagnostic difficulties. We report a nine-year-old boy with recurrent mass of his right thigh. Tumor cells were round, with scant cytoplasm, finely dispersed chromatin, and inapparent, small nucleoli.

Neonatal pulmonary sequestration (PS) with rhabdomyomas-like hyperplasia: A case report.

Introduction: Pulmonary sequestration (PS) is a rare pulmonary congenital malformation characterized by disconnection with the tracheobronchial tree or the pulmonary arterial blood supply thus impeding the connection to the arterial blood supply from systemic circulation, ultimately resulting in a non-functional lung.

Patient Concerns: A 73-day-old boy with rhabdomyomatoid hyperplasia was hospitalized for cough and fever 2 months after birth.

Diagnoses: Routine B-ultrasound revealed a cystic malformation in the right lung.

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.

Bronchiolitis obliterans organizing pneumonia due to gastroesophageal reflux.

The most common causes of bronchiolitis obliterans organizing pneumonia (BOOP) are connective tissue diseases, organ transplantation, drug reaction, and infections. Although rare, BOOP due to gastroesophageal reflux (GER) has been reported in adults but not to date in pediatric patients. This study describes 2 pediatric patients who developed GER and BOOP.

[Molecular genetic abnormalities of N-myc and C-myc in pediatric neuroblastic tumors and clinical pathologic significance].

Objective: To investigate the molecular genetic abnormalities of N-myc and C-myc, and their clinical pathological implications in pediatric neuroblastic tumors (NTs).

Methods: Abnormalities of N-myc were detected by interphase fluorescence in situ hybridization (FISH) technique in 246 cases of NTs, including neuroblastoma (NB,188 cases), ganglioneuroblastoma (GNB, 52 cases), ganglioneuroma (GN, 6 cases), and their association with the histological typing of the tumors and prognosis was analyzed. Abnormalities of C-myc were detected by FISH in 133 cases of NTs.

[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.

[Primitive neuroectodermal tumor/Ewing's sarcoma of the penis in children: a case report and review of the literature].

Objective: To investigate the clinical manifestations, pathological characteristics and treatment of primitive neuroectodermal tumor/Ewing's sarcoma (PNET/EWS) of the penis in children.

Methods: We analyzed the clinical data of a case of PNET/EWS and reviewed relevant literature.

Results: The patient was a 5-year-old boy, admitted for penis swelling with pain for 11 months.

[N-myc gene copies in children with neuroblastoma and its clinical significance].

This study was purposed to analyze the relation of N-myc gene copy number with clinical staging, pathological types and tumor biological factors in children with neuroblastoma (NB), and to investigate the influence of chemotherapy on N-myc gene expression and explore the relationship of N-myc gene copies with prognosis of NB children. The newly diagnosed children with NB from 1 March 2007 to 31 January 2011 were enrolled in this study. The treatment was carried out by BCH-NB-2007 based on Hongkong NB-07 protocol, and the patients were follow up to 31 January 2012.

[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 and laboratory characteristics of systemic anaplastic large cell lymphoma in Chinese patients.

Background: Systemic anaplastic large cell lymphoma (S-ALCL) is a rare disease with a highly variable prognosis and no standard chemotherapy regimen. Anaplastic lymphoma kinase (ALK) has been reported as an important prognostic factor correlated with S-ALCL in many but not all studies. In our study, we retrospectively analyzed 92 patients with S-ALCL from the Peking University Lymphoma Center for clinical and molecular prognostic factors to make clear the role of ALK and other prognostic factors in Han Chinese S-ALCL.

[Clinical features of 3 cases with acute interstitial pneumonia in children].

Objective: Acute interstitial pneumonia (AIP) is a rare lung interstitial disease in children. This study was conducted to understand the clinical features of the AIP in children.

Method: The data of the three cases with AIP admitted to our hospital from March 2008 to November 2009 were reviewed.

[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 characteristics of 5 children with Castleman's disease and review of literature].

Objective: To investigate the clinical characteristics of children with Castleman's disease and to improve doctors' awareness of this disease.

Methods: Clinical characteristics of 5 cases with Castleman's disease were observed and analyzed and relevant reports in literature were reviewed.

Results: (1) All the five patients' histories were long, and the first symptoms of them were painless lymphnode enlargement, and all of them were at school age; 3 patients' abdominal lymphnodes were enlarged, mediastinum lymphnodes enlarged in 3 cases, cervical lymphnodes were involved in 3 cases; (2) The clinical subtypes: the disease in 3 cases was localized Castleman's disease (LCD), all of their pathological subtype was hyaline vascular variant (HV).

[Idiopathic interstitial pneumonias in 7 children].

Objective: Idiopathic interstitial pneumonias (IIPs) have been increasing in children in recent years. The type and prognosis of IIPs in children in China has not been clear. The purpose of this study was to investigate the type and prognosis of IIPs in children.

[Congenital intrapulmonary lymphangioma and pulmonary hemangioma in 5 infants and young children].

Objective: To analyze the clinical, imaging and pathological findings of congenital intrapulmonary lymphangioma and hemangioma in 5 infants and young children.

Method: Data of 3 cases with congenital intrapulmonary lymphangioma and 2 cases with haemangioma were analyzed.

Result: All the 5 cases had cough, difficulty in breathing, cyanosis of lips, and shortness of breath.

[Detection of C-terminal 30 bp-deletion mutation of latent membrane protein 1 of EBV in childhood lymphoma].

Objective: To study C-terminal 30 bp-deletion mutation of latent membrane protein 1 of the virus from childhood lymphoma.

Methods: Nested-PCR was used to amplify C-terminal of EBV-LMP1 from childhood lymphoma and non-lymphoma associated to EBV, including Hodgkin lymphoma (HL), non-Hodgkin lymphoma (NHL)and reactive hyperplasia of lymph node (RL). Sequence analysis was performed on the positive PCR product.

[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.