Clinical features of the children with malignancy-associated hemophagocytic syndrome in Beijing / 中华实用儿科临床杂志

Objective@#To investigate the clinical features, treatment and prognosis of the children with malignancy-associated hemophagocytic syndrome (MAHS) in Beijing in recent decade.@*Methods@#The clinical data of the patients with MAHS under 18 years old from July 2007 to February 2018 collected by the Society of Beijing hemophagocytic syndrome were analyzed retrospectively.@*Results@#There were 46 patients under 18 years old with MAHS in all(male 27, female 19). The patients with MAHS took up 8.9% of the patients with hemophagocytic lymphohistiocytosis (HLH)(46/519 cases) from the area during that period.The median age of onset had 13.5 years (0.9-18.0 years). Thirty-five patients had lymphoma (76.0%), 9 cases had leukemia (19.6%), 1 case had myelodysplastic syndrome with refractory anemia with excess blast(RAEB-T), and 1 case had Epstein-Barr virus(EBV) associated lymphoproliferative disease (borderline tumor stage). All the patients had a fever.A half of them suffered from hepatosplenomegaly and 7 patients(15.2%) had neurological symptoms.The common laboratory abnormalities included cytopenias, hemophagocytosis in bone marrow (81.8%, 36/44 cases), elevated serum ferritin (87.8%, 36/41 cases), and elevated sCD25 (100.0%, 15/15 cases), decreased nature killer(NK) activity (61.1%, 11/18 cases), and plasma EBV-DNA positive (57.9%). Four patients did not receive treatment, the rest were treated by several chemotherapy protocols including the HLH-94/2004 protocol.Five patients (10.8%) received the allogeneic hematopoietic stem cell transplantation, and 1 case received the splenectomy therapy.The mortality was 58.7%.Four heterozygous mutations of SH2D1A, UNC13D and PRF1 genes were found in 2 patients.@*Conclusions@#MAHS in children progresses rapidly, with poor prognosis, and it is often complicated with EBV infection.The children with MAHS may develop genetic defects similar to primary hemophagocytic syndrome.Nowadays, there is no standard treatment for MAHS, so the individualized treatment is to be explored.

Clinical features of the children with malignancy-associated hemophagocytic syndrome in Beijing / 中华实用儿科临床杂志

Objective To investigate the clinical features,treatment and prognosis of the children with malignancy-associated hemophagocytic syndrome (MAHS) in Beijing in recent decade.Methods The clinical data of the patients with MAHS under 18 years old from July 2007 to February 2018 collected by the Society of Beijing hemophagocytic syndrome were analyzed retrospectively.Results There were 46 patients under 18 years old with MAHS in all(male 27,female 19).The patients with MAHS took up 8.9％ of the patients with hemophagocytic lymphohistiocytosis (HLH) (46/519 cases) from the area during that period.The median age of onset had 13.5 years (0.9-18.0 years).Thirty-five patients had lymphoma (76.0％),9 cases had leukemia (19.6％),1 case had myelodysplastic syndrome with refractory anemia with excess blast (RAEB-T),and 1 case had Epstein-Barr virus (EBV) associated lymphoproliferative disease (borderline tumor stage).All the patients had a fever.A half of them suffered from hepatosplenomegaly and 7 patients (15.2％) had neurological symptoms.Tbe common laboratory abnormalities included cytopenias,hemophagocytosis in bone marrow (81.8％,36/44 cases),elevated serum ferritin (87.8％,36/41 cases),and elevated sCD25 (100.0％,15/15 cases),decreased nature killer (NK) activity (61.1％,11/18 cases),and plasma EBV-DNA positive (57.9％).Four patients did not receive treatment,the rest were treated by several chemotherapy protocols including the HLH-94/2004 protocol.Five patients (10.8％) received the allogeneic hematopoietic stem cell transplantation,and 1 case received the splenectomy therapy.The mortality was 58.7％.Four heterozygous mutations of SH2D1A,UNC13D and PRF1 genes were found in 2 patients.Conclusions MAHS in children progresses rapidly,with poor prognosis,and it is often complicated with EBV infection.The children with MAHS may develop genetic defects similar to primary hemophagocytic syndrome.Nowadays,there is no standard treatment for MAHS,so the individualized treatment is to be explored.

Advances in hereditary research of primary hemophagocytic lymphohistiocytosis / 国际儿科学杂志

Hemophagocytic lymphohistiocytosis(HLH)is a life-threatening syndrome characterized by multiple organs infiltrations of lymphocytes and histiocytes with proliferation and hemophagocytic activity,and pancytopenia.HLH can be classified as either primary or secondary.Primary HLH includes familial HLH and immune deficiency syndromes associated HLH.Until now, familial HLH is classified into five types, whereas,immune deftciencv svndromes associated HLH is divided into Chediak-Higashi syndrome, GrisceUi syndrome 2,research of primary HLH. screening genetic defects and researches in functions of target genes and coding proteins, the effect of these genes and proteins in pathogenesis of HLH could be known further.

EFFECT OF CHLOROGENIC ACID ON GLUCOSE CONSUMPTION IN HepG2 CELLS PRETREATED WITH HIGH INSULIN AND HIGH OLEIC ACID / 营养学报

Objective To explore the modulation of chlorogenic acid (CGA) on glucose metabolism in HepG2 cells pretreated with high insulin and high oleic acid (OA). Methods Cultured HepG2 cells induced by high insulin and oleic acid for insulin resistance and steatosis respectively, were co-cultured with different concentrations of CGA (10,20,40,80 mg/L) for 24h. The morphological changes were observed and glucose consumptions of cells were measured by glucose oxidase method. Results Compared to control group, CGA could significantly increase glucose consumption of normal HepG2 cells and the dosedependent effect was noted between 10-40 mg/L(P