Pentoxifyline versus prednisolone for severe alcoholic hepatitis: A randomized controlled trial

AIM: To compare the efficacy of pentoxifylline and prednisolone in the treatment of severe alcoholic hepatitis, and to evaluate the role of different liver function scores in predicting prognosis. METHODS: Sixty-eight patients with severe alcoholic hepatitis (Maddrey score ≥ 32) received pentoxifylline ( n = 34, group Ⅰ) or prednisolone ( n = 34, group Ⅱ) for 28 d in a randomized double-blind controlled study, and subsequently in an open study (with a tapering dose of prednisolone) for a total of 3 mo, and were followed up over a period of 12 mo. RESULTS: Twelve patients in group Ⅱ died at the end of 3 mo in contrast to five patients in group Ⅰ. The probability of dying at the end of 3 mo was higher in group Ⅱ as compared to group Ⅰ (35.29% vs 14.71%, P = 0.04; log rank test). Six patients in group Ⅱ developed hepatorenal syndrome as compared to none in group Ⅰ. Pentoxifylline was associated with a significantly lower model for end-stage liver disease (MELD) score at the end of 28 d of therapy (15.53 Maddrey score was associated with increased mortality. CONCLUSION: Reduced mortality, improved risk-benefit profile and renoprotective effects of pentoxifylline compared with prednisolone suggest that pentoxifylline is superior to prednisolone for treatment of severe alcoholic hepatitis.     

Clinical characteristics and prognosis of young patients with colorectal cancer in Eastern China

AIM:To explore the clinical characteristics and prognosis of young patients with colorectal cancer patients in Eastern China.METHODS:A total of 1335 patients with colorectal cancer treated from December 1985 to December 2005at the Second Affiliated Hospital of Zhejiang University School of Medicine were studied retrospectively.The patients were divided into two groups,a younger group(aged≤30 years)and an older group(aged>30 years),and comparison was made in the clinical characteristics and prognosis between the two groups.Chisquare test was used for data analysis of all categorical variables,and overall survival(OS)was calculated by the Kaplan-Meier method.A multivariate analysis was performed using the Cox model.RESULTS:There were 42(3.1%)and 1293(96.9%)cases in the younger group and older group,respectively.Univariate analysis showed that the 5-and10-year OS in the younger group were 33.9%and26.1%,respectively,and those in the older group were60.1%and 52.2%,respectively.Younger group had poor survival(χ2=14.146,P=0.000).Multivariate analysis revealed that age was not a dependent factor for prognosis(OR=0.866,95%CI:0.592-1.269,P=0.461).Stratified analysis indicated that in stageⅢandⅣdisease,the 5-and 10-year OS were 24.6%and14.8%in the younger group,and 40.4%and 33.3%in the older group,respectively,with a significant difference between the two groups(χ2=5.101,P=0.024).In the subgroup of radical surgery,the 5-and 10-year OS were 44.3%and 34.2%in the younger group,and69.6%and 60.5%in the older group,with a difference being significant between the two groups(χ2=7.830,P=0.005).CONCLUSION:Compared with older patients,the younger patients have lower survival,especially in the subgroups of stageⅢandⅣdisease and radical surgery.     

Prognostic roles of preoperative α-fetoprotein and des-γ-carboxy prothrombin in hepatocellular carcinoma patients

AIM:To clarify the utility of using des-γ-carboxy prothrombin(DCP)andα-fetoprotein(AFP)levels to predict the prognosis of hepatocellular carcinoma(HCC)in patients with hepatitis B virus(HBV)and the hepatitis C virus(HCV)infections.METHODS:A total of 205 patients with HCC(105patients with HBV infection 100 patients with HCV infection)who underwent primary hepatectomy between January 2004 and May 2012 were enrolled retrospectively.Preoperative AFP and DCP levels were used to create interactive dot diagrams to predict recurrence within 2 years after hepatectomy,and cutoff levels were calculated.Patients in the HBV and HCV groups were classified into three groups:a group with low AFP and DCP levels(LL group),a group in which one of the two parameters was high and the other was low(HL group),and a group with high AFP and DCP levels(HH group).Liver function parameters,the postoperative recurrence-free survival rate,and postoperative overall survival were compared between groups.The survival curves were compared by logrank test using the Kaplan-Meier method.Multivariate analysis using a Cox forward stepwise logistic regression model was conducted for a prognosis.RESULTS:The preoperative AFP cutoff levels for recurrence within 2 years after hepatectomy in the HBV and HCV groups were 529.8 ng/m L and 60 m AU/m L,respectively;for preoperative DCP levels,the cutoff levels were 21.0 ng/m L in the HBV group and 67 m AU/m L in the HCV group.The HBV group was significantly different from the other groups in terms of vascular invasion,major hepatectomy,volume of intraoperative blood loss,and surgical duration.Significant differences were found between the LL group,the HL group,and the HH group in terms of both mean disease-free survival time(MDFST)and mean overall survival time(MOST):64.81±7.47 vs 36.63±7.62 vs 18.98±6.17mo(P=0.001)and 85.30±6.55 vs 59.44±7.87 vs46.57±11.20 mo(P=0.018).In contrast,the HCV group exhibited a significant difference in tumor size,vascular invasion,volume of intraoperative blood loss,and surgical duration;however,no significant difference was observed between the three groups in liver function parameters except for albumin levels.In the LL group,the HL group,and the HH group,the MDFST was 50.09±5.90,31.01±7.21,and 14.81±3.08 mo(log-rank test,P0.001),respectively,and the MOST was 79.45±8.30,58.82±7.56,and 32.87±6.31 mo(log-rank test,P0.001),respectively.CONCLUSION:In the HBV group,the prognosis was poor when either AFP or DCP levels were high.In the HCV group,the prognosis was good when either or both levels were low;however,the prognosis was poor when both levels were high.High levels of both AFP and DCP were an independent risk factor associated with tumor recurrence in the HBV and HCV groups.The relationship between tumor marker levels and prognosis was characteristic to the type of viral hepatitis.     

Clinical prognostic factors for disabling Crohn’s disease: A systematic review and meta-analysis

AIM: To identify demographic and clinical factors asso-ciated with disabling Crohn’s disease (CD). METHODS: A systematic review and meta-analysisof observational studies, focusing on the factors that can predict the prognosis of different outcomes of CD was undertaken. PubMed, ISI Web of Knowledge and Scopus were searched to identify studies investigat-ing the above mentioned factors in adult patients with CD. Studies were eligible for inclusion if they describe prognostic factors in CD, with inclusion and exclusion criteria defined as follows. Studies with adult patients and CD, written in English and studying association between clinical factors and at least one prognosis out-come were included. Meta-analysis of effects was un-dertaken for the disabling disease outcome, using odds ratio (OR) to assess the effect of the different factors in the outcome. The statistical method used was Mantel-Haenszel for fixed effects. The 16-item quality assess-ment tool (QATSDD) was used to assess the quality of the studies (range: 0-42). RESULTS: Of the 913 papers initially selected, sixty studies were reviewed and three were included in the systematic review and meta-analysis. The global QA-TSDD scores of papers were 18, 21 and 22. Of a total of 1961 patients enrolled, 1332 (78%) were classified with disabling disease five years after diagnosis. In two studies, age at diagnosis was a factor associated with disabling disease five years after diagnosis. Individu-als under 40 years old had a higher risk of developing disabling disease. In two studies, patients who were treated with corticosteroids on the first flare developed disabling disease five years after diagnosis. Further, perianal disease was found to be relevant in all of the studies at two and five years after diagnosis. Finally, one study showed localization as a factor associated with disabling disease five years after diagnosis, with L3 being a higher risk factor. This meta-analysis showed a significantly higher risk of developing disabling dis-ease at five year     

Crohn’s-like acute severe colitis associated with Hermansky-Pudlak syndrome: A case report

BACKGROUND Hermansky-Pudlak syndrome(HPS)is a rare autosomal recessive disorder characterized by oculocutaneous albinism,platelet storage pool deficiency and systemic complications associated with ceroid deposition in the reticuloendothelial system.HPS types 1 and 4 are associated with Crohn’s disease(CD)-like gastrointestinal disorders,such as granulomatous enterocolitis or perianal disease.Cases of colitis can be particularly severe and,before the use of anti-tumor necrosis factor alpha(TNFα)therapy had become common,were reported as showing poor responsiveness to medical treatment.CASE SUMMARY We present the case of a 51-year-old albino woman who presented with acute severe colitis that led to the diagnosis of HPS.Histologic findings of biopsy samples showed chronic inflammation with deep ulcerations,and granulomas without caseous necrosis.Molecular genetic analysis confirmed HPS type 1,with a homozygous 27 base-pair deletion in exon 20 of the HPS1 gene.Once the patient’s bleeding diathesis was corrected by platelet transfusion,the granulomatous colitis responded dramatically to a medical treatment regimen that included corticosteroids,azathioprine and infliximab;this regimen is similar to that used in CD treatment.Although it remains unclear if the granulomatous enterocolitis in HPS is due to ceroid deposition or reflects the co-existence of CD and HPS,the fact that this case of HPS-related granulomatous colitis responded to the same therapeutic approach used in CD suggests that this type of colitis may result from HPS patients’genetic susceptibility to CD.CONCLUSION We report a case of severe colitis that led to the diagnosis of HPS,which was responsive to azathioprine and infliximab.     

Proton-pump inhibitors for prevention of upper gastrointestinal bleeding in patients undergoing dialysis

AIM:To investigate the preventive effects of low-dose proton-pump inhibitors(PPIs) for upper gastrointestinal bleeding(UGIB) in end-stage renal disease.METHODS:This was a retrospective cohort study that reviewed 544 patients with end-stage renal disease who started dialysis at our center between 2005 and 2013.We examined the incidence of UGIB in 175 patients treated with low-dose PPIs and 369 patients not treated with PPIs(control group).RESULTS:During the study period, 41 patients developed UGIB, a rate of 14.4/1000 person-years.The mean time between the start of dialysis and UGIB events was 26.3 ± 29.6 mo.Bleeding occurred in only two patients in the PPI group(2.5/1000 person-years) and in 39 patients in the control group(19.2/1000 person-years).Kaplan-Meier analysis of cumulative non-bleeding survival showed that the probability of UGIB was significantly lower in the PPI group than in the control group(log-rank test, P 0.001).Univariate analysis showed that coronary artery disease, PPI use, anti-coagulation, and anti-platelet therapy were associated with UGIB.After adjustments for the potential factors influencing risk of UGIB, PPI use was shown to be significantly beneficial in reducing UGIB compared to the control group(HR = 13.7, 95%CI:1.8-101.6; P = 0.011).CONCLUSION:The use of low-dose PPIs in patients with end-stage renal disease is associated with a low frequency of UGIB.     

Significance of antineutrophil cytoplasmic antibody in adult patients with Henoch-Schnlein purpura presenting mainly with gastrointestinal symptoms

AIM: To test the clinical significance of antineutrophil cytoplasmic antibody (ANCA) in evaluation of adult Henoch-Schnlein purpura (HSP) patients presenting mainly with abdominal symptoms. METHODS: Twenty-eight consecutive HSP patients who presented predominantly with abdominal symptoms were enrolled in this study. Control subjects included 27 age-and sex-matched patients with peptic ulcer disease,colon cancer,acute gastroenteritis,irritable bowel syndrome and colonic polyps. ANCA was measured by indirect immunofluorescence (ⅡF) in all patients,and follow-up ELISA was performed in patients with positive ⅡF tests. RESULTS: ANCA was detected in 9 HSP patients by ⅡF (2 were positive for c-ANCA and 7 were positive for p-ANCA). No ANCA was found in the control group. The sensitivity and specifi city of a positive ANCA test (either c-or p-ANCA) were 32.1% and 100% respectively. Only one out of the 9 patients with positive ANCA by ⅡF had positive ANCA by ELISA and the antigen was myeloperoxidase (MPO). The patients positive for ANCA had higher HSP clinical scores,and were more likely to have renal function impairment. Patients with late purpura development were also associated with more severe clinical manifestations. CONCLUSION: A positive ANCA test is associated with more severe symptoms in HSP. After inflammatory bowel disease is excluded,a positive ANCA test provides a clue to the diagnosis of HSP presenting predominantly with abdominal symptoms.     

Echocardiography evaluation of right ventricular structure and function in patients with chronic obstructive pulmonary disease

Background Early and accurate assessment of right ventricular changes in patients with Chronic obstructive pulmonary disease(COPD)is of great value,because these changes are important factors affecting the prognosis of patients with COPD. This study aimed to investigate the changes of right ventricular structure and function by electrocardiography in COPD patients,and to evaluate the correlations of pulmonary function,COPD assessment test(CAT)score with the changes. Methods According to the"ABCD"2016 Global initiative for chronic obstructive pulmonary disease(GOLD)update,stable COPD patients were divided into four groups:GOLD A group,GOLD B group,GOLD C group,and GOLD D group. There were 30 patients of each group enrolled from December 2016 to December 2017 in Guangdong General Hospital. During the same period,30 physical examination volunteers were enrolled as the healthy control group. Pulmonary function and echocardiography were performed in all subjects. All COPD patients completed CAT test. One-way ANOVA and SNK-q test were used to analyze the differences in parameters of right ventricular structure and function between different GOLD groups and healthy controls. Pearson correlation analysis was used to analyze the relation between CAT score,forced expiratory volume in one second(FEV1)and echocardiographic parameters. Results Compared with control group,right ventricular wall thickness(RVWT)was significantly thicker in patients of GOLD A,B and C groups(P0.001),but not for GOLD D group. In sharp contrast,only in the patients of GOLD D group,right ventricular basal diameter(RV basal diameter)and right ventricular outflow tract(RVOT)(2.84±0.56 cm and 4.63±0.54 cm)were significantly longer than those in healthy controls and other GOLD groups(P0.001).The patients of GOLD C and D groups had higher E/A ratio and E/e′ ratio compared with control group,GOLD A and B groups(P0.001). But only in GOLD D group,the fractional area change(FAC)and tricuspid annular plane systolic excursion(TAPSE)were decreased significantly,compared with control group and other GOLD groups(P0.001). The right ventricular index of myocardial performance(RIMP)in GOLD B,C and D groups was increased significantly compared with healthy control and GOLD A group(P0.001). Moreover,correlation analysis showed that no correlations were found between FEV1 and all parameters of right ventricular structure and function,but CAT was positively correlated with RIMP(r=0.696,P=0.019). Conclusions Echocardiography could be a good assessment for right ventricular structure and function in COPD patients. Right ventricular wall thickening and diastolic dysfunction occur in the early stage of COPD,followed by right ventricular volume enlargement and systolic dysfunction.     

Follow-up of patients with functional bowel symptoms treated with a low FODMAP diet

AIM: To investigate patient-reported outcomes from, and adherence to, a low FODMAP diet among patients suffering from irritable bowel syndrome and inflammatory bowel disease.METHODS: Consecutive patients with irritable bowel syndrome(IBS) or inflammatory bowel disease(IBD) and co-existing IBS fulfilling the ROME Ⅲ criteria, who previously attended an outpatient clinic for low FODMAP diet(LFD) dietary management and assessment by a gastroenterologist, were invited to participate in a retrospective questionnaire analysis. The questionnaires were sent and returned by regular mail and gathered information on recall of dietarytreatment, efficacy, symptoms, adherence, satisfaction, change in disease course and stool type, and quality of life. Before study enrolment all patients had to sign an informed written consent.RESULTS: One hundred and eighty patients were included, 131(73%) IBS and 49(27%) IBD patients. Median age was 43 years(range: 18-85) and 147(82%) were females. Median follow-up time was 16 mo(range: 2-80). Eighty-six percent reported either partial(54%) or full(32%) efficacy with greatest improvement of bloating(82%) and abdominal pain(71%). The proportion of patients with full efficacy tended to be greater in the IBD group than in the IBS group(42% vs 29%, P = 0.08). There was a significant reduction in patients with a chronic continuous disease course in both the IBS group(25%, P 0.001) and IBD group(23%, P = 0.002) along with a significant increase in patients with a mild indolent disease course of 37%(P 0.001) and 23%(P = 0.002), respectively. The proportion of patients having normal stools increased with 41% in the IBS group(P 0.001) and 66% in the IBD group(P 0.001). One-third of patients adhered to the diet and high adherence was associated with longer duration of dietary course(P 0.001). Satisfaction with dietary management was seen in 83(70%) IBS patients and 24(55%) IBD patients. Eightyfour percent of patients lived on a modified LFD, where some foods rich in FODMAPs were reintroduced, and 16% followed the LFD by the book without deviations. Wheat, dairy products, and onions were the foods most often not reintroduced by patients.CONCLUSION: These data suggest that a diet low in FODMAPs is an efficacious treatment solution in the management of functional bowel symptoms for IBS and IBD patients.     

Genetic polymorphism of UL144 open reading frame of human cytomegalovirus DNA detected in colon samples from infants with Hirschsprung''''s disease

AIM:To explore the genetic diversities of UL144 open reading frame (ORF) of cytomegalovirus DNA detected in colon tissue from infants with Hirschsprung's disease (HD) by sequencing UL144 DNA in 23 aganglionic colon tissue and 4 urine samples from 25 HD infants. METHODS:Nest PCR was performed for amplification of the UL144 gene. The UL144 gene was analyzed with softwares,such as DNAclub,BioEdit,PROSITE database,and DNAstar. RESULTS:The strains from HD patients were distributed among three genotypes of UL144:group 1A (64%),group 2 (24%),and group 3 (12%). The UL144 genotypes between strains from HD and control group were compared by chi square test (χ2 = 1.870,P = 0.393). Strains from the colon were sporadically distributed in UL144 genotypes. CONCLUSION:There are genetic diversities of UL144 ORF in colon tissue of infants with HD. However,cytomegalovirus UL144 genotypes are not associated with clinical manifestations of HD.     

自身免疫病合并噬血细胞综合征临床分析

目的 通过分析11例自身免疫病(AID)合并噬血细胞综合征(HPS)的临床特点,提高对该病的认识.方法 收集北京协和医院2004年1月-2009年6月住院的AID合并HPS者11例,回顾性分析其发生诱因、各系统受累表现、治疗及预后.结果 11例AID合并HPS患者中男性3例,女性8例,年龄12 74(30.7±18.3)岁.基础病:斯蒂尔病4例,系统性红斑狼疮(SLE)3例,干燥综合征(SS)、类风湿关节炎(RA)、韦格纳肉芽肿(WG)、克罗恩病(CD)各1例.诱发HPS因素:基础病活动4例,基础病活动并发感染6例,单纯感染1例.11例患者均有高热,其中肝脾肿大8例,淋巴结肿大7例,神经系统受累4例,并发弥漫性血管内凝血(DIC)4例.实验室检查:血细胞减少11例,肝功能异常11例,高甘油三酯血症5例,低纤维蛋白原血症9例,铁蛋白＞500 μg/L 6例,NK细胞活性降低4例.骨髓涂片均见吞噬血细胞现象.经大剂量糖皮质激素联合免疫抑制剂、积极抗感染、静脉人免疫球蛋白(IVIG)支持治疗,5例存活,6例死亡,合并DIC 4例均未存活(r=0.69,P=0.019).结论 AID并发HPS不易与活动性AID鉴别.合并DIC预后差、病死率高.糖皮质激素、免疫抑制剂及IVIG对HPS治疗有效,合并感染时加强抗感染治疗至关重要.     

Clinical analysis of macrophage activation syndrome in pediatric patients with autoimmune diseases

Macrophage activation syndrome (MAS) belongs to secondary hemophagocytic lymphohistiocytosis (HLH) syndrome. It is usually associated with rheumatic diseases. We retrospectively reviewed our hospital's medical records of 102 HLH/MAS patients from the past 20 years. Demographics, clinical data, treatment, and outcomes were analyzed. Among 102 patients, eight patients with underlying juvenile systemic lupus erythematous (two patients), mixed connective tissue disease (one patient), primary anti-phospholipid syndrome (one patient), and systemic type juvenile rheumatoid arthritis (sJRA; four patients) with 13 episodes of MAS were studied. Clinical manifestations of MAS included fever (100 %), hepatosplenomegaly (77 %), lymphadenopathy (38 %), skin rash (62 %), and neurological involvement (31 %). Laboratory features included leukopenia (54 %), anemia (46 %), thrombocytopenia (77 %), jaundice (27 %), hypofibrinogenemia (40 %), decreased erythrocyte sedimentation rate (67 %), and elevated liver enzymes (77 %), lactate dehydrogenase (100 %), ferritin (88 %), triglycerides (91 %), C-reactive protein (85 %), plasma D-dimer (50 %), and hemophagocytosis in bone marrow (83 %). The Epstein-Barr virus and adenovirus infection triggered MAS in two patients with sJRA. Methylprednisolone pulse therapy was effective in two out of three patients, and high-dose intravenous immunoglobulin (IVIG) was effective in two out of six patients. Patients with sJRA responded well to corticosteroids and cyclosporine. Complications included opportunistic infection with Pneumocystis jiroveci, multiple organ failure, and intensive care unit myopathy. The mortality rate was one out of eight (12.5 %). Our results showed that MAS could be fatal and complicate various pediatric autoimmune diseases. It generally has a good response to corticosteroids and IVIG. Prompt recognition and timely treatment can result in good outcomes.     

Intraocular inflammation in autoimmune diseases

BACKGROUND: The uveal tract represents the vascular organ of the eye. In addition to providing most of the blood supply to the intraocular structures, it acts as a conduit for immune cells, particularly lymphocytes, to enter the eye. Consequently, the uveal tract is represented in many intraocular inflammatory processes. Uveitis is probably a misnomer unless antigens within the uvea are the direct targets of the inflammatory process. A better term of the condition is "intraocular inflammation" (IOI). OBJECTIVES: To review the presence of IOI in autoimmune diseases, the immunopathogenic mechanisms leading to disease, and treatment. METHODS: We reviewed the English medical literature by using MEDLINE (1984-2003) employing the terms "uveitis," "intraocular inflammation," and "autoimmune diseases." RESULTS: An underlying autoimmune disease was identified in up to 40% of patients with IOI, and included spondyloarthropathies, Behcets disease, sarcoidosis, juvenile chronic arthritis, Vogt-Koyanagi-Harada syndrome (an inflammatory syndrome including uveitis with dermatologic and neurologic manifestations), immune recovery syndrome, and uveitis with tubulointerstitial disease. The immunopathogenesis of IOI involves enhanced T-cell response. Recently, guidelines for the use of immunosuppressive drugs for inflammatory eye disease were established and include: corticosteroids, azathioprine, methotrexate, mycophenolate mofetil, cyclosporine, tacrolimus, cyclophosphamide, and chlorambucil. New therapies with limited experience include the tumor necrosis factor alpha inhibitors, interferon alfa, monoclonal antibodies against lymphocyte surface antigens, intravenous immunoglobulin (IVIG), and the intraocular delivery of immunosuppressive agents. CONCLUSION: An underlying autoimmune disease was identified in up to 40% of patients with IOI. Immunosuppressive drugs, biologic agents, and IVIG are employed for the treatment of IOI in autoimmune diseases.     

Allogeneic stem cell transplantation for Evans syndrome.

Evans syndrome is a rare disorder characterized by combined autoimmune thrombocytopenia (ITP) and autoimmune hemolytic anemia (AIHA). Standard treatments consist of transfusions, corticosteroids, splenectomy, IVIG, anabolic steroids, vincristine, alkylating agents, or cyclosporine. In a patient with refractory disease, an allogeneic hematopoietic stem cell transplant (HSCT) resulted in complete clinical and serologic remission for more than 30 months. Allogeneic HSCT may be the only current curative therapy for Evans syndrome but may also be complicated by significant toxicities.     

Hemophagocytic Lymphohistiocytosis: Clinical Analysis of 103 Adult Patients

To investigate the clinical features of adult patients with hemophagocytic lymphohistiocytosis (HLH) and to explore possible risk factors for death, we retrospectively reviewed the medical records of 103 adult HLH patients hospitalized from 1997 to 2012. We analyzed the underlying diseases, clinical characteristics, 1aboratory findings, outcomes, and prognostic factors. The most common cause of HLH was hematologic malignancies (n = 49), followed by infectious diseases (n = 24) and autoimmune disorders (n = 14); 24 cases were of unknown etiology. Eight patients had a combination of underlying diseases. HLH was clinically characterized by high fever (96.1%), splenomegaly (79.6%), hepatomegaly (65.0%), lymphadenopathy (53.4%), proteinuria (31.1%), skin rash (25.2%), gastrointestinal hemorrhage (14.6%), disseminated intravascular coagulation (13.6%), increased creatinine (7.8%), and central nervous system involvement (12.6%) including altered mental status (9.7%) and cranial hemorrhage (2.9%). Laboratory abnormalities included cytopenia (99.0%), serum ferritin >500 ug/L (98.4%), liver dysfunction (98.1%), hypertriglyceridemia (88.5%), hemophagocytosis in bone marrow smear (87.4%), and hypofibrinogenemia (60.9%).In addition to the treatment they received for the underlying causes, patients received therapy for HLH consisting of corticosteroids, immunosuppressive drugs, and intravenous immunoglobulin. Twenty-six patients (25.2%) recovered after treatment, and 19 of them achieved long-term remission during follow-up. Seventy-seven patients (74.8%) died because of tumor, sepsis, multiple organ failure, or HLH-related organ hemorrhage and coagulopathy. The deceased patients were more likely to be older at disease onset, male, and to present with splenomegaly and thrombocytopenia, compared to the survivors. Treatment for the underlying diseases combined with corticosteroids, immunosuppressive agents, and immunoglobulin therapy may improve the prognosis of HLH. More attention should be paid to high-risk patients to prevent the development of serious complications associated with HLH.Key words/Abbreviations: hemophagocytic lymphohistiocytosis, lymphoma, autoimmune diseases, clinical manifestation, prognosis, risk factor, AOSD = adult-onset Still disease, CNS = central nervous system, DIC = disseminated intravascular coagulation, CMV = cytomegalovirus, EBV = Epstein-Barr virus, HLH = hemophagocytic lymphohistiocytosis, IVIg = intravenous immunoglobulin, MAS = macrophage-activation syndrome, NK = natural killer, RA = rheumatoid arthritis, SLE = systemic lupus erythematosus     

Therapeutic responses and prognosis in adult-onset Still’s disease

To date, the treatment of adult-onset Still’s disease (AOSD) has been largely empirical; therefore, this study was conducted to investigate the response to therapy and prognostic factors of AOSD. Fifty-four Korean patients with AOSD were enrolled based on Yamaguchi’s criteria. We retrospectively analyzed the treatments and prognosis. Thirty-nine patients (72.2%) were female, and the average age at disease onset was 37.3 years. Twenty-nine patients had a monocyclic disease (53.7%), five had a polycyclic (9.3%) and fifteen had a chronic articular disease (27.7%) and five died (9.3%). The elevated ESR and corticosteroids refractoriness were associated with poor prognosis (P = 0.023 and P = 0.009, respectively). The patients that died were older than those survived (49.2 ± 11.8 vs. 42.2 ± 14 year old, P = 0.024). Forty-two patients were treated with non-steroidal anti-inflammatory drugs; however, they also needed corticosteroids and intravenous immunoglobulin (IVIG). Among 50 patients treated with high-dose corticosteroids, 21 patients (42%) were resistant to corticosteroids and treated with IVIG or anti-tumor necrosis factor (TNF) agents. Of the 23 patients medicated with IVIG, the prognosis was better in IVIG-responsive patients, indicating a therapeutic effect. Methotrexate was the most commonly used disease modifying anti-rheumatic drugs (27 patients, 50%), and the corticosteroid requirements were lower in the methotrexate-responsive patients. Approximately half of AOSD patients had a poor prognosis and were corticosteroids resistance. An elevated ESR and non-response to corticosteroids were associated with poor prognosis. Patients who died were older than those survived.     

Hemophagocytic syndrome

INTRODUCTION: Hemophagocytic syndrome results from a inappropriate stimulation of macrophages in bone marrow and lymphoid organs, leading to phagocytosis of blood cells and production of high amounts of pro-inflammatory cytokines. This life-threatening disease combines non-specific clinical signs (fever, cachexia, hepatomegaly, enlargement of spleen and lymph nodes) as well as typical laboratory findings (bi- or pancytopenia, abnormal hepatic tests, hypofibrinemia, elevation of serum LDH, ferritinemia and triglyceride levels). Diagnosis is confirmed by cytological or pathological examination of bone marrow or tissue specimens. Hemophagocytosis may be primitive, essentially in pediatric population, or secondary, related to various situations such as lymphomas, infections (viral, bacterial or parasitic) or auto-immune diseases. Prognosis is poor, depending on the associated disease, with an overall mortality of 50%. CURRENT KNOWLEDGE AND KEY POINTS: Recent advances, essentially due to genetic studies of familial hemophagocytic syndrome, have underlined the major role of T lymphocytes and TNF alpha in the pathogenesis of hemophagocytosis. In these pediatric cases, prognosis has dramatically improved since allogenic bone marrow transplantation is performed, raising long-term survival from 10 to 66%. FUTURE PROSPECTS AND PROJECTS: In secondary forms of hemophagocytic syndrome, treatment must be symptomatic (transfusion, correction of electrolyte disorders) and etiological (chemotherapy, anti-viral or antibiotic drugs, immunosuppressive therapy). However, prospective trials are necessary to define the best treatment in these cases. New therapeutic options, targeting specific mediators, including TNF alpha, may emerge with the understanding of pathogenesis of hemophagocytic syndrome.     

Alternative Therapies for Neonatal Autoimmune Thrombocytopenia

Objective: This study was performed to test whether corticosteroids were superior to intravenous immunoglobulins (IVIG) in the treatment of neonatal autoimmune thrombocytopenia (NAT). Methods: All cases received IVIG, and unresponsive cases received corticosteroids. Results: Of 7 babies who received IVIG, only 1 responded. The 6 remaining cases received corticosteroids therafter, and all of them responded well to this therapy. Conclusions: Corticosteroids may be more effective than IVIG in NAT.     

A case of hemophagocytic syndrome with terminal ileal ulcerations]

Reactive hemophagocytic syndrome or hemophagocytic lymphohistiocytosis, is characterized by the proliferation of benign histiocytes showing phagocytosis of blood cells in hematopoietic organs including bone marrow, spleen, or lymph nodes, accompanied by fever, hepatosplenomegaly, hepatic dysfunction, pancytopenia, and hypertriglyceridemia. The pathogenesis of reactive hemophagocytic syndrome is unknown. It is often associated with infection, malignant neoplasm, autoimmune disease, drugs and various immunodeficiencies. The prognosis of this syndrome is poor and the causes of death are hemorrhage, infection, or multiorgan failure. We experienced a case of hemophagocytic syndrome with terminal ileal ulcers, not associated with other causes. Thus, we report this case with a review of literatures.     

噬血细胞综合征12例临床分析

目的探讨噬血细胞综合征的临床表现、诊断及治疗方法。方法对12例患者进行回顾性分析。结果12例患者中感染相关性8例，肿瘤相关性3例，原因不明1例。治愈6例，好转1例，死亡5例。结论根据临床症状，体征和实验室检查，骨髓细胞学检查，多可获得诊断，对于治疗应主要针对病因，应用肾上腺糖皮质激素，由恶性疾病引起及病因未明者预后较差。