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Patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) have a poor prognosis. This study investigated the efficacy of rituximab-containing immuno-chemotherapy regimens for EBV-HLH. In this study, 15 patients were treated with rituximab-containing regimens. The treatment efficacy and adverse events were evaluated. In 10 patients, EBV DNA became negative after the first course of treatment. The lymphocyte types infected by EBV in the 10 patients were only infected with B cells and mainly infected with B cells. In the other 5 patients, the EBV DNA of peripheral blood mononuclear cells (PBMC) before and after treatment with the regimens had no statistical difference (P = 0.111). In addition, in these 5 patients, EBV mainly infected T and NK cells. Among the 5 patients without a significant decline in EBV DNA of PBMC, 2 patients received allogeneic hematopoietic stem cell transplantation and turned negative for EBV DNA. This study suggests that rituximab-containing regimens are effective therapy for EBV-HLH with only and mainly B lymphocytes infected by EBV, especially for eliminating EBV., (Copyright © 2021. Published by Elsevier B.V.)

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.

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., (© 2018 S. Karger AG, Basel.)

Leprosy is an infectious chronic granulomatous disease caused by Mycobacterium leprae. The disease mainly affects the skin, peripheral nerves, mucosa, and viscera. The World Health Organization has reported that most countries with high endemicity have reached the goal of eliminating leprosy (defined as reaching a prevalence of <1 leprosy case per 10 000 population) at the national level, after years of proactive control campaigns. The incidence of leprosy has been decreasing across the globe year by year. However, misdiagnosis happens occasionally due to the complexity of clinical manifestations and lack of physician awareness of this disease. We report a case of lepromatous leprosy complicated by hemophagocytosis misdiagnosed as hemophagocytic lymphohistiocytosis., (Copyright © 2014 The Authors. Published by Elsevier Ltd.. All rights reserved.)

The study was aimed to investigate the etiology and the clinical characteristics of patients with hemophagocytic syndrome. The clinical data of 38 patients with hemophagocytic syndrome were retrospectively analyzed, and prf1 and stx11 were detected for the mutational analysis. The results showed that 38 cases were diagnosed as hemophagocytic syndrome, including 1 case of familial hemophagocytic lymphohistiocytosis (FHL), 14 cases associated with infectious disease (36.84%), 10 cases with malignancies (26.32%), 7 cases with rheumatic disease (18.42%), other 6 cases of unknown etiology (15.79%). 9 out of 38 cases died with mortality of 23.68%, including 4 cases associated with infectious disease, 2 cases with malignancies, 1 case with rheumatic disease, and 2 cases of unknown etiology. One case was found to have prf1 mutation, and was diagnosed as FHL at last. It is concluded that the causes of HPS are diverse, different etiology results in different outcome. It is important to find etiology when HPS is diagnosed, and prf1 and stx11 genetic analysis plays a important role in the diagnosis of FHL.

The aim of study was to establish an accurate and stable technique for the detection of NK cell activity in the diagnosis of hemophagocytic lymphohistiocytosis (HLH). 21 suspected acquired HLH patients and 20 healthy subjects as controls were enrolled in the study, and the suspected HLH patients were divided into confirmedly diagnosed group and excluded group according to HLH-2004 diagnostic criteria. The plasmid pEGFP-N1 was transfected into K562 cells. After scanned with G4l8 and monoclone, the EGFP-K562 cell line stably expressing enhanced green fluorescent protein was obtained. PBMNC and EGFP-K562 were mixed at the effector to target ratio of 10:1. After incubation for of 2 hours, propidium iodine (PI) was added to stain dead cells, and then cytotoxic activity was analyzed by using flow cytometry. Meanwhile, the cytotoxic activity of NK cells in peripheral blood on K562 cells was detected by LDH release assay, and was compared with results detected by flow cytometry. The results showed that a K562 cell line stably expressing EGFP was constructed and were used to measure NK cytotoxicity against the target cells by flow cytometry without pre-staining or pre-labeling target cells. There was significant difference in killing rate of NK cells between the diagnostic group and the control group. This new technique correlated strongly with the results by LDH release assay. In conclusion, this study provides a novel, simple, rapid, repeatable and reproducible method to measure NK activity by flow cytometry using EGFP-K562 without pre-staining or pre-labeling target cells. It may be widely used in the diagnosis of hemophagocytic lymphohistiocytosis.

The aim of this study was to investigate the clinical features of acquired hemophagocytic lymphohistiocytosis (HLH) complicated with hepatic dysfunction. 18 cases of acquired HLH were analyzed. The characteristics of hepatic dysfunction, the relationship between hepatic dysfunction and the cause, as well as prognosis of the acquired HLH were preliminarily analysed. The results indicated that characteristics of hepatic dysfunction in acquired HLH patients were hypoproteinemia, jaundice and increase of L-aspirate aminotransferase (AST) and lactate dehydrogenase (LDH) levels. The level of AST and direct bilirubin (DBil) in the non-malignancy associated hemophagocytic lymphohistiocytosis group were higher than that in malignancy-associated hemophagocytic lymphohistiocytosis group (p<0.05). And the increase of LDH and AST levels indicated poor prognosis (p<0.05). In conclusion, liver damage is a common organ functional disorder in patients with acquired HLH, which may be correlated to the cause and the prognosis of acquired HLH.

The study was aimed to investigate the clinical characteristics, diagnosis and therapy of patients with lymphoma associated hemophagocytic syndrome (LAHS) so as to provide the clinical basis for improving its recognition and giving effective therapy. The clinical data of 14 patients with LAHS in Beijing Friendship Hospital, Capital Medical University during the period from June 2005 to May 2008 were collected, the informations including primary diseases, clinical manifestations, laboratory findings, therapy and outcome were analyzed retrospectively, the coincidence of each diagnostic index was compared before and after diagnosis. All 14 patients were given therapeutic regimens containing fludarabine, methylprednisolone and gammaglobulin (FDIg) after final diagnosis. The results indicated that 100% patients had abnormal changes on NK cell activity and sCD25 level in serum, but hemophagocytosis in less than 40% patients at early stage was found in bone marrow. Even after confirmed diagnosis of the disease, the percentage of patients with hemophagocytosis was not up to 50%. 9 out of the 14 patients had a good prognosis after treatment, and the other 5 patients died. It is concluded that the detection of NK cell activity and sCD25 level in serum may be valuable for the early diagnosis of LAHS, the hemophagocytosis is not necessary for the diagnosis of LAHS. Fludarabine combined with methylprednisolone and gamma globulin may provide a new strategy for LAHS therapy.

The study was aimed to explore the diagnostic significance of hemophagocytosis in the patients with hemophagocytic lymphohistiocytosis (HLH). 61 suspected HLH patients from June 2005 to October 2008 were enrolled in the study. The suspected HLH patients were divided into confirmed group (43 out of 61) and excluded group (18 out of 61) according to HLH-2004 diagnostic criteria. The incidences of hemophagocytosis in bone marrow, spleen or lymph nodes were compared in all groups. The results indicated that the hemophagocytosis in bone marrow, spleen or lymph nodes was found in 33 patients of confirmed group, while the hemophagocytosis was found in 4 patients of excluded group. The sensitivity of hemophagocytosis for the diagnosis of HLH was 76.7%, and its specificity was 77.8%. In conclusion, hemophagocytosis is a helpful marker for the diagnosis of most but not all HLH patients, however, the lack of hemophagocytosis does not mean to exclude the diagnosis of HLH.

This study was aimed to explore the level of glycosylated ferritin in the patients with secondary hemophagocytic lymphohistiocytosis (HLH) and its diagnostic significance. 29 suspected HLH patients from October 2007 to October 2008 were enrolled in the study, and 25 healthy subjects were selected as control. The 29 suspected HLH patients were divided into confirmed group (22 out of 29) and unconfirmed group (7 out of 29) according to HLH-2004 diagnostic criteria. The percentage of glycosylated ferritin in peripheral blood was determined by phytohemagglutinin adsorption assay. The results showed that the median level of total serum ferritin in patients of confirmed group (2897.6+/-1837.2 microg/L) was significantly higher than that in patients of unconfirmed group (653.1+/-249.1 microg/L) (p<0.01), and was also higher than that in controls (414.6+/-212.6 microg/L) (p<0.01). The median percentage of glycosylated ferritin in patients of confirmed group was significantly lower (17.0+/-4.2%) than that in patients of unconfirmed group (40.7+/-4.5%) (p<0.01) and was lower than that in controls (53.6+/-13.3%) (p<0.01). The sensitivity (86.4% vs 77.3%) and specificity (71.4% vs 42.9%) of glycosylated ferritin for the diagnosis of HLH were higher than that of total serum ferritin. In conclusions, glycosylated ferritin may be a helpful marker for the diagnosis of HLH.