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Haemophagocytic lymphohistiocytosis (HLH) is a rare and severe clinical syndrome that can be classified as either primary or secondary. Secondary HLH can be triggered by a variety of diseases. Pregnancy-related HLH has already been observed clinically. However, most of these cases occur during pregnancy. Considering that the periods before and after delivery have different clinical features, we presented the first case series of HLH that presented during the postpartum stage of pregnancy. From these cases, we concluded that postpartum HLH is a common form of pregnancy-related HLH. Patients with postpartum HLH may suffer more complicated pathogenesis. Cytopenia was not as common as in other types of HLH, but liver dysfunction was present in almost all cases. The standard therapy of HLH-94/04 was very effective, and the outcomes of postpartum HLH were better. [ABSTRACT FROM AUTHOR]

Objective: To investigate the drug resistant related FOXO3/Bcl-6 signaling pathway in K562/G cell line and its related microRNA(miRNA) mechanisms., Methods: The drug resistance potency of imatinib on K562/G was detected by MTT assay. The expression of FOXO3 and Bcl-6 proteins in K562 and K562/G cells was detected by Western blot. Real-time PCR (RT-PCR) was used to detect the expression of FOXO3 and Bcl-6 mRNA. The miRNA expression profiling in K562 and K562/G cells was analyzed by microarray technique, and the miRNA targeted to FOXO/Bcl-6 signaling pathway was identified., Results: The expression of FOXO3 and Bcl-6 protein was significantly increased in K562/G cells as compared with that in K562 cells (P<0.01), the expression level of Bcl-6 mRNA showed no increase in K562/G cells. However, FOXO3 mRNA was up-regulated in K562/G cells (P<0.05). MiRNA microarray results showed that 109 miRNAs were expressed differentially in K562 and K562/G cells. The expression of 81 miRNAs were up-regulated while 28 miRNAs were down-regulated. Through reverse prediction by bioinformatics, miR-6718-5p, miR-5195-5p, miR-4711-3p, miR-4763-5p, miR-4664-5p and miR-3176 were related to FOXO/Bcl-6 signaling pathway., Conclusion: The FOXO3/Bcl-6 signaling pathway contributes to imatinib resistance in K562/G cell line, and the miRNA expression profiles showed significant differences between K562/G and K562 cells.

Objective: To establish a secondary hemophagocytic lymphohistiocytosis(HLH) mouse model, and to investigate the effect of ruxolitinib on the disease manifestation of model mice., Methods: Wild type C57BL/6 mice were randomly divided into 4 groups: two groups of mice were intraperitoneally injected with CpG oligodeoxynucleotide 1826 (CpG-ODN1826) every other day to induce HLH, and other two groups were control groups. One group of the CpG-ODN1826 groups and one of the control groups were given ruxolitinib, and other two groups were given the same amount of PBS. Blood samples, serum ferritin and hepatic/spleen weights of experimental mice were detected and serum cytokine levels were measured by ELISA., Results: Compared with the control groups, the levels of white blood cells, hemoglobin and platelets in the CpG-ODN1826 groups were significantly lower (P＜0.05); and liver/body weight, spleen/body weight, serum ferritin, sCD25, IL-10, IL-1β, IFN-Ƴ, IL-12p70, GM-CSF, TNF-α and IL-18 levels significantly increased (P＜0.05). There was no significant difference in the levels of IL-2, IL-4, IL-5, IL-6, IL-22, IL-13, IL-27 and IL-23 between the two groups (P＞0.05). The spleen in CpG group had disordered internal structure, expanding red pulp and hyperplastic nucleated cells. The liver had severe perivascular inflammations. The spleen/weight of the ruxolitinib-treated mice in the CpG-ODN1826 group was significantly smaller than that of the unapplied ruxolitinib (P＜0.05)., Conclusion: The CpG-ODN1826 can induce secondary HLH symptoms in wild type C57BL/6 mice. Ruxolitinib can alleviate the symptoms of splenomegaly in HLH model mice.

Objective: To detect the erythrocyte life by breath carbon monoxide assay and to analyze the effect of erythrocyte life on the anemia in patients with hemophagocytic syndrome., Methods: The breath carbon monoxide test and erythrocyte life assay were performed for 20 cases of hemophagocytic syndrome, at the same time the detection of 20 healthy persons was used as control. The difference of anemia-related indexes was compared between hemophagocytic syndrome patients and healthy persons., Results: The average erythrocyte life of patients with hemophagocytic syndrome was 45.3 days, which was 65% shorter than that of healthy persons. Hemoglobin levels positively correlated with erythrocyte life, while sCD25, breath carbon monoxide concentration and hemophagocytosis negatively correlated with erythrocyte life. Bilirubin level and reticulocyte count showed no correlation with erythrocyte life. Serum level of IL-1β, IL-2, sCD25, IL-6, IL-10, IL-17A, GM-CSF, TNF-α and IFN-γ in patients with newly diagnosed hemophagocytic syndrome were significantly higher than those of healthy persons (P＜0.05)., Conclusion: Breath carbon monoxide assay is a rapid and efficient method to assess erythrocyte life. Increased erythrocyte destruction may be an important pathogenic factor for anemia in hemophagocytic syndrome.

Objective: To investigate the clinical characteristics, therapeutic outcomes and prognostic factors of primary central nervous system lymphoma (PCNSL)., Methods: Clinical records of 31 cases of PCNSL were collected, the clinical charactenstics were analyzed retrospectively. Survival curves were estimated using Kaplan-Meier survival methodology and statistical significance of continuous variables was assessed via the Cox proportional hazard model., Results: The median age was 52 years, with a ratio of male to female 1:1. Headache/dizzy/limb numbness were the most common presentation and the lesions of PCNSL were primarily located at the frontal, parietal, temporal lobes and corpus callosum. All the cases were pathologically diffuse large B cell lymphoma (DLBCL), 6 cases were the type of germinal center (GC) and 25 cases of non-GC type, after craniotomy operation and biopsy. Among 31 cases, 17 patients accepted the combined treatment, 3 patients underwent simple chemotherapy, 8 patients received simple radiotherapy, the other patients accepted support therapy. The median follow-up for surviving patients was 24 months. The 1-, 3-, and 5-year survival rates were 80.6%, 55.1%, and 36.4%, respectively. The median overall survival time in the combined treatment group was significantly longer than that in simply radiotherapy. There was no significant difference in OS between the groups with and without rituximab. ECOG PS≥2 and elevated serum LDH predicted inferior survival., Conclusion: The clinical manifectation of PCNSL is various and complicated, and for the time being there is no optimal treatment scheme. The overall survival time of the combined treatment is longer than that in simply radiotherapy. ECOG PS≥2 and elevated serum LDH often are poor prognostic factors.

Erdheim-Chester Disease (ECD) is a rare form of non Langerhans' cell histiocytosis. The etiology of ECD is unknown yet. The disease is characterized by the infiltration of lipid-laden histiocytes with foamy to bones and various organs. The heterogeneous manifestations of ECD vary among different individuals. This results in a presentation that may vary from an indolent focal disease to a life threatening organ failure. This review focuses on the etiology, clinical presentation, diagnostic criteria and treatment of Erdheim-Chester disease (ECD).