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“Typhoidal Cells”Appear in a Woman with Hemophagocytic Syndrome Secondary To Brucellosis:A Case Report
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作者 Wei-Qing Song Xu Zheng +3 位作者 Hai-Ni Li Li Li Jiang-Shui Yuan Shu-Guo Wang 《Chinese Medical Sciences Journal》 CAS CSCD 2023年第1期62-65,共4页
We report a case of hemophagocytic syndrome(HPS)secondary to brucellosis,in which typhoidal cells were found in bone marrow,suggesting typhoidal cells present not only in Salmonella typhi infections but also in other ... We report a case of hemophagocytic syndrome(HPS)secondary to brucellosis,in which typhoidal cells were found in bone marrow,suggesting typhoidal cells present not only in Salmonella typhi infections but also in other bacterial infections.Typhoidal cells in bone marrow can be used to quickly identify the presence of bacterial infection pending the results of bone marrow and/or blood cultures. 展开更多
关键词 BRUCELLOSIS hemophagocytic syndrome typhoidal cells
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Epstein–Barr virus-associated hemophagocytic syndrome in a patient with ulcerative colitis during treatment with azathioprine: A case report and review of literature 被引量:3
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作者 Kazuya Miyaguchi Minoru Yamaoka +7 位作者 Yoshikazu Tsuzuki Keigo Ashitani Hideki Ohgo Yoshitaka Miyagawa Keisuke Ishizawa Hidekazu Kayano Hidetomo Nakamoto Hiroyuki Imaeda 《World Journal of Clinical Cases》 SCIE 2018年第14期776-780,共5页
A 19-year-old female was diagnosed with ulcerative colitis when she presented with persistent melena, and has been treated with 5-aminosalicylic acid for 4 years, with additional azathioprine for 2 years at our hospit... A 19-year-old female was diagnosed with ulcerative colitis when she presented with persistent melena, and has been treated with 5-aminosalicylic acid for 4 years, with additional azathioprine for 2 years at our hospital. The patient experienced high-grade fevers, chills, and cough fve d prior to presenting to the outpatient unit. At frst, the patient was suspected to have developed neutropenic fever; however, she was diagnosed with Epstein-Barr virus-associated hemophagocytic syndr-ome (EB-VAHS) upon fulfilling the diagnostic criteria after bone marrow aspiration. When patients withinflammatory bowel disease treated with immunomo-dulators, such as thiopurine preparations, develop fever, EB-VAHS should be considered in the differential diagnosis. 展开更多
关键词 Infammatory bowel disease AZATHIOPRINE Virus-associated hemophagocytic syndrome Ulcerative colitis Case report
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Clinical and Biological Significance of Clonal Macrophage Detection in Hemophagocytic Syndrome 被引量:1
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作者 LIN Wen(林雯) +3 位作者 XIAO Yan(肖燕) FEI Hongbao(费洪宝) 《Journal of Huazhong University of Science and Technology(Medical Sciences)》 SCIE CAS 2002年第2期126-128,共3页
Summary: By using the method of clonal analysis the evidence to prove that Hemophagocytic syndrome (HPS) is reactive or malignant was investigated to probe into the pathogenesis of HPS and its relations with clinical ... Summary: By using the method of clonal analysis the evidence to prove that Hemophagocytic syndrome (HPS) is reactive or malignant was investigated to probe into the pathogenesis of HPS and its relations with clinical prognosis. The macrophages abnormally proliferated in bone marrow were isolated. Electrophoresis analysis was made after DNA extraction, enzyme restriction of human ardrogen receptor (HUMARA) genetic locus, and PCR amplification. In the 9 specimens, clonal proliferation was found in 2 cases and nonclonal proliferation in 7. Among the 7 cases of nonclonal proliferation, 3 were voluntarily discharged without clinical outcome, 2 cases fully recovered after 2-3 week treatment of large dose gamma globulin intravenous drip and hormone therapy, 1 case died at the 43th day after the hormone and anti-infection therapy, and one case was found to have granular leukoblast in peripheral blood after 3 weeks and diagnosed as having M2a after bone puncture. For the two patients with clonal proliferation, one obtained remission after chemotherapy and the other was died after 32 days without chemotherapy. It was concluded that there do exist clonal or malignant proliferation in HPS, so not every case is reactive. 展开更多
关键词 hemophagocytic syndrome CLONE CHEMOTHERAPY
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Diagnostic value of bone marrow cell morphology in visceral leishmaniasis-associated hemophagocytic syndrome:Two case reports 被引量:2
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作者 Shu-Lan Shi Heng Zhao +4 位作者 Beng-Jiang Zhou Ming-Biao Ma Xiao-Juan Li Ji Xu Hong-Chao Jiang 《World Journal of Clinical Cases》 SCIE 2022年第16期5463-5469,共7页
BACKGROUND Visceral leishmaniasis related-hemophagocytic lymphohistiocytosis(VL-HLH)is a hemophagocytic syndrome caused by Leishmania infection.VL-HLH is rare,especially in nonendemic areas where the disease is severe... BACKGROUND Visceral leishmaniasis related-hemophagocytic lymphohistiocytosis(VL-HLH)is a hemophagocytic syndrome caused by Leishmania infection.VL-HLH is rare,especially in nonendemic areas where the disease is severe,and mortality rates are high.The key to diagnosing VL-HLH is to find the pathogen;therefore,the Leishmania must be accurately identified for timely clinical treatment.CASE SUMMARY We retrospectively analyzed the clinical data,laboratory examination results,and bone marrow cell morphology of two children with VL-HLH diagnosed via bone marrow cell morphology at Kunming Children’s Hospital of Yunnan,China.Both cases suspected of having malignant tumors at other hospitals and who were unresponsive to treatment were transferred to Kunming Children’s Hospital.They are Han Chinese girls,one was 2 years old and the other one is 9 mo old.They had repeated fevers,pancytopenia,hepatosplenomegaly,hypertriglyceridemia,and hypofibrinogenemia over a long period and met the HLH-2004 criteria.Their HLH genetic test results were negative.Both children underwent chemotherapy as per the HLH-2004 chemotherapy regimen,but it was ineffective and accompanied by serious infections.We found Leishmania amastigotes in their bone marrow via morphological examination of their bone marrow cells,which showed hemophagocytic cells;thus,the children were diagnosed with VL-HLH.After being transferred to a specialty hospital for treatment,the condition was well-controlled.CONCLUSION Morphological examination of bone marrow cells plays an important role in diagnosing VL-HLH.When clinically diagnosing secondary HLH,VL-HLH should be considered in addition to common pathogens,especially in patients for whom HLH-2004 chemotherapy regimens are ineffective.For infants and young children,bone marrow cytology examinations should be performed several times and as early as possible to find the pathogens to reduce potential misdiagnoses. 展开更多
关键词 Bone marrow cell morphology Visceral leishmaniasis hemophagocytic syndrome INFANT Case report
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Infection-associated Hemophagocytic Syndrome in Critically Ill Patients with COVID-19 被引量:2
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作者 Kun YANG Ming-you XING +10 位作者 Ling-yu JIANG Yan-ping CAI Li-li YANG Na-na XIE Jia CHEN Wen-xia WANG Li WANG Ji-ling ZHU Ding-yu ZHANG Qiu-rong RUAN Jian-xin SONG 《Current Medical Science》 SCIE CAS 2021年第1期39-45,共7页
Infection-associated hemophagocytic syndrome(IAHS),a severe complication of various infections,is potentially fatal.This study aims to determine whether IAHS occurs in critically ill patients with coronavirus disease ... Infection-associated hemophagocytic syndrome(IAHS),a severe complication of various infections,is potentially fatal.This study aims to determine whether IAHS occurs in critically ill patients with coronavirus disease 2019(COVID-19).We conducted a retrospective observational study on 268 critically ill patients with COVID-19 between February 1st,2020 and February 26th,2020.Demographics,clinical characteristics,laboratory results,information on concurrent treatments and outcomes were collected.A diagnosis of secondary hemophagocytic lymphohistiocytosis(sHLH)was made when the patients had an HScore greater than 169.Histopathological examinations were performed to confirm the presence of hemophagocytosis.Of 268 critically ill patients with confirmed SARS-CoV-2 infection,17(6.3%)patients had an HScore greater than 169.All the 17 patients with sHLH died.The interval from the onset of symptom of COVID-19 to the time of a diagnosis of sHLH made was 19 days and the interval from the diagnosis of sHLH to death was 4 days.Ten(59%)patients were infected with only SARS-CoV-2.Hemophagocytosis in the spleen and the liver,as well as lymphocyte infiltration in the liver on histopathological examinations,was found in 3 sHLH autopsy patients.Mortality in sHLH patients with COVID-19 is high.And SARS-CoV-2 is a potential trigger for sHLH.Prompt recognition of IAHS in critically ill patients with COVID-19 could be beneficial for improving clinical outcomes. 展开更多
关键词 severe acute respiratory coronavirus 2 coronavirus disease 2019 infection-associated hemophagocytic syndrome
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Unexpected Occurrence of Fetal Hemophagocytic Syndrome in a Patient with Hereditary Diffuse Leukoencephalopathy with Spheroids 被引量:2
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作者 Yasufumi Kondo Michiaki Kinoshita +6 位作者 Takuhiro Yoshida Hisanori Matoba Takeshi Uehara Meguru Ikeyama Jun Nakayama Kunihiro Yoshida Shu-Ichi Ikeda 《Case Reports in Clinical Medicine》 2016年第3期77-84,共8页
Colony stimulating factor-1 receptor (CSF1R) plays important roles in the differentiation and proliferation of macrophage and microglia in systemic organs and the brain. A genetic defect in CSF1R causes hereditary dif... Colony stimulating factor-1 receptor (CSF1R) plays important roles in the differentiation and proliferation of macrophage and microglia in systemic organs and the brain. A genetic defect in CSF1R causes hereditary diffuse leukoencephalopathy with spheroids (HDLS). HDLS mainly affects the cerebral white matter and shows pre-senile cognitive decline, motor disturbance, and epilepsy. However, systemic manifestations outside the brain have not yet been described in patients with HDLS. Here, we report the case of a 41-year-old man with HDLS carrying the p. K793T mutation in CSF1R, who unexpectedly died of sepsis and hemophagocytic syndrome shortly after the onset of HDLS. The fetal sequence of sepsis and hemophagocytic syndrome was triggered by enterocolitis. An autopsy revealed that focal inflammation in the intestine had almost resolved. Most strikingly, massive infiltration of cluster of differentiation (CD) 68- and CD163-immunopositive macrophages with hemophagocytosis was observed in the bone marrow, spleen, and liver. Less abundant infiltration of CD68- and CD204-immunopositive macrophages without hemophagocytosis was also seen in the lung and intestine. At present, the pathogenetic link between CSF1R mutation and hemophagocytic syndrome in this patient is unclear. Our case, however, clearly shows that even in patients with HDLS, aberrant activation of functional macrophages can be induced under certain conditions in visceral organs. 展开更多
关键词 HDLS (Hereditary Diffuse Leukoencephalopathy with Spheroids) CSF1R (Colony Stimulating Factor-1 Receptor) ENTEROCOLITIS Macrophage hemophagocytic syndrome
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CLINICAL STUDY ON 15 CASES OF HEMOPHAGOCYTIC SYNDROME
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作者 王宝燕 郑成云 +3 位作者 陈学民 李刚 韩云峰 刘陕西 《Journal of Pharmaceutical Analysis》 CAS 1996年第1期80-82,90,共4页
cases of hemophagocytic syndrome (HS) have been investigated in recent 5 years. we found that the underlying causes of HS existed broadly including virus infections (hepatitis virus,cytomegalovirus, influenza),bacteri... cases of hemophagocytic syndrome (HS) have been investigated in recent 5 years. we found that the underlying causes of HS existed broadly including virus infections (hepatitis virus,cytomegalovirus, influenza),bacteria infections(tubercle bacillus, pneumococcus,bacillus pyocyaneus,etc.),fungus infection and tumors (multiple myeloma,lymphomas).The major manifestations were as follows: fever, fatigue, lymphadenovarix,hematosplenomegaly,pancytopenia in peripheral blood, histocyte hyperlasia and histocytosis in bone marrow.7 HS cases with nonfatal infection and tumors recovered. In order to improve the correct diagnosis level and decrease mortality of HS, we discussed the possible mechanism of the onset of HS and its underlying causes. 展开更多
关键词 hemophagocytic syndrome CLINICAL STUDY
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A case of hemophagocytic syndrome as a complication of <i>Plasmodium</i>vivax malaria
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作者 Ananya Mukharjee Chintan Sagar +2 位作者 Sahil Fulara Uday Patil Monika Jawanjal 《Open Journal of Clinical Diagnostics》 2013年第4期156-158,共3页
Hemophagocytic lymphohistiocytosis syndrome (HPS) is a potentially fatal hyperinflammatory response characterized by a generalized histiocytic proliferation with marked hemophagocytosis in bone marrow [1]. Hemophagocy... Hemophagocytic lymphohistiocytosis syndrome (HPS) is a potentially fatal hyperinflammatory response characterized by a generalized histiocytic proliferation with marked hemophagocytosis in bone marrow [1]. Hemophagocytic syndrome has been associated with genetic mutations, autoimmune diseases, hematological malignancies or infections [2,3]. According to the data from Centre for Disease Control and prevention (CDC) Plasmodium falciparum has been associated with HPS but not the Plasmodium vivax [4-7]. We report a case of hemophagocytic syndrome as a complication of Plasmodium vivax malaria which is a rare presentation according to the data. This patient presented with high grade fever with chills (P. vivax positive), fever however did not respond to anti-malarials. The patient continued to have high grade fever with altered sensorium and deranged liver function with pancytopenia. Since she fulfilled the criteria of (HPS), patient was put on injectable steroids and responded dramatically. Hemophagocytic syndrome is a potentially fatal syndrome and therefore high index suspicion and early treatment is the key to reduce the mortatlity. 展开更多
关键词 hemophagocytic syndrome PLASMODIUM VIVAX MALARIA
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Fatal hemophagocytic lymphohistiocytosis presenting as Reye’s syndrome: Report of two cases
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作者 Shinji Kounami Koichi Minami +3 位作者 Megumi Yoshiyama Genkichi Izumi Noriyuki Aoyagi Norishige Yoshikawa 《Open Journal of Pediatrics》 2012年第2期161-164,共4页
We report two cases of rapidly fatal hemophagocytic lymphohistiocytosis (HLH) that presented as Reye’s syndrome (RS). The patients were referred to our hospital because of altered level of consciousness with hyperamm... We report two cases of rapidly fatal hemophagocytic lymphohistiocytosis (HLH) that presented as Reye’s syndrome (RS). The patients were referred to our hospital because of altered level of consciousness with hyperammonemia and hypoglycemia. The first patient, a 24-month-old girl (Case 1), died soon after arrival and was clinically diagnosed as having RS, but a diagnosis of HLH was established on the basis of autopsy findings. The other patient, a 4-month-old boy (Case 2), was diagnosed as having HLH in view of the bone marrow findings on admission, but immunosuppressive therapy failed to prevent a rapidly fatal course. Marked hypercytokinemia was found in both patients, and liver pathology demonstrated panlobular microvesicular steatosis of hepatocytes, resembling that in RS. Mononuclear cell infiltration with hemophagocytosis in the liver was evident in case 2, but not in case 1. In both cases, hypercytokinemia was thought to have caused mitochondrial dysfunction, resulting in RS-like microvesicular steatosis of hepatocytes. HLH is an important differential diagnosis of children presenting with RS-like picture. 展开更多
关键词 hemophagocytic LYMPHOHISTIOCYTOSIS Reye’s syndrome CYTOKINE Mitochondria ENCEPHALOPATHY
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Sepsis complicated with secondary hemophagocytic syndrome induced by giant gouty tophi rupture: A case report
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作者 Bin Lai Zhi-Hui Pang 《World Journal of Clinical Cases》 SCIE 2022年第23期8224-8231,共8页
BACKGROUND Gouty tophi are a chronic granulomatous caused by a deposition of monosodium urate crystal deposition in the body.Once broken,it may easily induce severe infection.Sepsis complicated with secondary hemophag... BACKGROUND Gouty tophi are a chronic granulomatous caused by a deposition of monosodium urate crystal deposition in the body.Once broken,it may easily induce severe infection.Sepsis complicated with secondary hemophagocytic syndrome induced by gouty tophi rupture is extremely rare in the clinical setting,and no such serious complications have been reported in literature.CASE SUMMARY This is a 52-year-old Chinese male patient with a 20-year history of gouty arthritis.At admission,the gout stone in the patient’s right ankle was broken and it secreted a white mucoid substance.During the course of treatment,the patient suffered from systemic inflammatory response syndrome multiple times.His condition gradually deteriorated,further complicated by hemophagocytic syndrome.After thorough removal of gout lesions and active anti-infection treatment and control of blood uric acid level,combined with multidisciplinary cooperation,the patient was finally cured.CONCLUSION Sepsis complicated with secondary hemophagocytic syndrome induced by gouty tophi rupture is extremely rare in the clinical setting.Timely and accurate diagnosis is very important to save patients'lives. 展开更多
关键词 Gouty tophi SEPSIS Secondary hemophagocytic syndrome Multidisciplinary cooperation Case report
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Guillain-Barrésyndrome and hemophagocytic syndrome heralding the diagnosis of diffuse large B cell lymphoma:A case report
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作者 Qiao-Lin Zhou Zhao-Kun Li +4 位作者 Fang Xu Xiao-Gong Liang Xing-Biao Wang Jing Su Yu-Feng Tang 《World Journal of Clinical Cases》 SCIE 2022年第26期9502-9509,共8页
BACKGROUND Central nervous system(CNS)lesions and peripheral neuropathy are rare among patients with non-Hodgkin’s lymphoma(NHL).Lymphomatous infiltration or local oppression usually accounts for CNS or peripheral ne... BACKGROUND Central nervous system(CNS)lesions and peripheral neuropathy are rare among patients with non-Hodgkin’s lymphoma(NHL).Lymphomatous infiltration or local oppression usually accounts for CNS or peripheral nerve lesions.The incidence of peripheral neuropathy was 5%.Guillain-Barrésyndrome(GBS)is rare and may occur in less than 0.3%of patients with NHL.Hemophagocytic syndrome(HPS)is a rare complication of NHL.It has been reported that 1%of patients with hematological malignancies develop HPS.Diffuse large B-cell lymphoma(DLBCL)combined with GBS has been reported in 10 cases.CASE SUMMARY We report the case of a 53-year-old man who was initially hospitalized because of abnormal feelings in the lower limbs and urinary incontinence.He was finally diagnosed with DLBCL combined with GBS and HPS after 16 d,which was earlier than previously reported.Immunoglobulin pulse therapy,dexamethasone,and etoposide were immediately administered.The neurological symptoms did not improve,but cytopenia was relieved.However,GBS-related clinical symptoms were relieved partially after one cycle of rituximab-cyclophosphamide,hydroxydaunorubicin,vincristine,and prednisone(R-CHOP)chemotherapy and disappeared after six cycles of R-CHOP.CONCLUSION GBS and HPS heralding the diagnosis of Epstein-Barr virus DLBCL are rare.Herein,we report a rare case of DLBCL combined with GBS and HPS,and share our clinical experience.Traditional therapies may be effective if GBS occurs before lymphoma is diagnosed.Rapid diagnosis and treatment of DLBCL are crucial. 展开更多
关键词 Diffuse large B cell lymphoma Guillain-Barrésyndrome hemophagocytic syndrome Peripheral neuropathy Case report
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Toxoplasmosis-Associated Secondary Hemophagocytic Syndrome: A Case Report and Literature Review
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作者 Xiang Sun Jiakui Zhang +8 位作者 Qianling Ye Fan Wu Yinwei Li Qianshan Tao Qing Zhang Weiwei Zhu Huiping Wang Jinli Zhu Zhimin Zhai 《Open Journal of Blood Diseases》 2020年第3期61-70,共10页
<b style="line-height:1.5;"><span style="font-family:Verdana;">Background</span><span style="font-family:Verdana;">:</span></b><span style="fon... <b style="line-height:1.5;"><span style="font-family:Verdana;">Background</span><span style="font-family:Verdana;">:</span></b><span style="font-family:'';line-height:1.5;"><span style="font-family:Verdana;"> Toxoplasmosis is an extremely rare disease that occurs in the hosts contact with cat and dog frequently. Most human acute infections go unnoticed in immunocompetent individuals, and gradually transformed into chronic infection. However, while host immunity significantly waned, the risk of reactivation of chronic toxoplasma infection is greatly increased. Reactivation of latent toxoplasmic infection often presents with fever, leukopenia, thrombocytopenia, neurological signs and pneumonia. However, for the non-specific clinical and biological signs and its fetal outcome, toxoplasmosis is often misdiagnosed and only revealed at autopsy. </span><b><span style="font-family:Verdana;">Case Presenation</span></b></span><b style="line-height:1.5;"><span style="font-family:Verdana;">:</span></b><span style="line-height:1.5;font-family:Verdana;"> We report a case hospitalized for a week history of cough, anorexia and fatigue with nasal bleeding for a day. After hospitalization, broad-spectrum antibiotherapy was started without a clear diagnosis of infection. Then the patient did a lot of investigation</span><span style="line-height:1.5;font-family:Verdana;">s</span><span style="line-height:1.5;font-family:Verdana;"> to search the cause of infection, but there were no positive findings. However, an unexpected discovery was detected from peripheral blood smears, shows 1</span><span style="font-family:'';line-height:1.5;"> </span><span style="line-height:1.5;font-family:Verdana;">-</span><span style="font-family:'';line-height:1.5;"> </span><span style="line-height:1.5;font-family:Verdana;">3</span><span style="font-family:'';line-height:1.5;"> </span><span style="font-family:'';line-height:1.5;"><span style="font-family:Verdana;">μm in diameter, scattered, short and bow like microorganisms on Day 39 of hospitalization. Combined with the medical history and clinical manifestation, toxoplasmosis was diagnosed finally. Unfortunately, secondary hemophagocytic syndrome (HPS) was diagnosed only two days after targeted anti-infection therapy, and the patient died on Day 45 of hospitalization. </span><b><span style="font-family:Verdana;">Conclusions</span></b></span><b style="line-height:1.5;"><span style="font-family:Verdana;">:</span><span style="font-family:'';"> </span></b><span style="line-height:1.5;font-family:Verdana;">Patient with unexplained long-term fever and neurological symptoms, interstitial pneumonitis or myocarditis, toxoplasmosis should be positively considered. Only early diagnosis and treatment can increase the possibility of a successful cure and avoid other secondary diseases. 展开更多
关键词 TOXOPLASMOSIS INFECTION REACTIVATION hemophagocytic syndrome
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Kikuchi-Fujimoto Disease with Hemophagocytic Syndrome and Pleural Effusion in an Elder Man: A Case Report and Literature Review
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作者 Yanfang Zhang Jianchuan Deng 《Case Reports in Clinical Medicine》 2020年第10期311-318,共8页
<strong>Background:</strong> Kikuchi-Fujimoto disease (KFD), also named Histiocytic necrotizing lymphadenitis (HNL), is a rare and self-limited necrotizing lymphadenitis, most commonly affects young Asian ... <strong>Background:</strong> Kikuchi-Fujimoto disease (KFD), also named Histiocytic necrotizing lymphadenitis (HNL), is a rare and self-limited necrotizing lymphadenitis, most commonly affects young Asian female. KFD is characterized by lymphadenopathy, cytopenia, fever, rashes, and weight loss, however, hemophagocytic syndrome and pleural effusion are rarely reported. <strong>Case Presentation:</strong> We present the case of a 61-year-old man with clinical features including fever, lymphadenopathy, purpura, arthralgia, pleural effusion and pulmonary infection. He had cervical lymphnodebiopsy and the pathology is consistent with KFD, laboratory tests and clinical manifestations were consistent with hemophagocytic syndrome (HLH). After initial treatment including corticosteroid and anti-infection drugs, the patient showed improvement but soon deteriorated. <strong>Conclusion:</strong> KFD is a self-limited disease with spontaneous resolution. However, the cases of KFD with HLH may have a fatal course. Early appropriate intensive immunosuppressive therapy and strong anti-infection therapy could be beneficial for the treatment outcome. 展开更多
关键词 Kikuchi-Fujimoto Disease hemophagocytic syndrome Pleural Effusion
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Drug associated vanishing bile duct syndrome combined with hemophagocytic lymphohistiocytosis
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作者 Hui Li Xin Li +5 位作者 Xiao-Xing Liao Hong Zhan et al. Yan Xiong Chun-Lin Hu Hong-Yan Wei Xiao-Li Jing 《World Journal of Gastrointestinal Endoscopy》 2012年第8期376-378,共3页
A 28-year-old woman with untreated autoimmune disorder, demonstrated skin rash and fever after taking Amoxicillin-clavulanate and developed progressive jaundice. A bone marrow aspiration indicated an increased number ... A 28-year-old woman with untreated autoimmune disorder, demonstrated skin rash and fever after taking Amoxicillin-clavulanate and developed progressive jaundice. A bone marrow aspiration indicated an increased number of macrophages with hemophagocytosis and liver biopsy showed pure centrilobular cholestasis with necrosis and some absence of portal bile ducts. Furthermore, a serological test for Epstein-Barr virus was positive. Under treatment by liver dialysis and administration of steroids led to rapidly defervescence and clinical improvement. However, liver enzymes were still markedly elevated with persistent anemia, even after immunosuppressive treatment. The patient is currently waiting for liver transplantation. This is the ?rst description of vanishing bile duct syndrome combined with hemophagocytic lymphohistiocytosis, with underlying causes including infection, drug-induced factors and untreated autoimmune disorder. 展开更多
关键词 Vanishing bile duct syndrome hemophagocytic lymphohistiocytosis Epstein-Barr virus Amoxicillin-clavulanate
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One Hemophagocytic Syndrome Relating to Adult Epstein-Barr Virus Infection and a Literature Review
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作者 Xiaolong Liu Yamin Zhang +2 位作者 Zilin Cui Qing Tian Dongming Duan 《Case Reports in Clinical Medicine》 2016年第12期511-517,共7页
Background: Adult hemophagocytic syndrome is a critical condition that is often difficult to diagnose and results in a bad prognosis because of the lack of effective and unified treatment. Aim: To investigate the clin... Background: Adult hemophagocytic syndrome is a critical condition that is often difficult to diagnose and results in a bad prognosis because of the lack of effective and unified treatment. Aim: To investigate the clinical, diagnosis and treatment of hemophagocytic syndrome. Case Presentation: A 22-year-old female patient received a splenectomy, and the spleen was sent to the King Med Center of Medical Inspection. The results confirmed the diagnosis of hemophagocytic syndrome related to EB (Epstein-Barr) virus infection. Conclusion: Early diagnosis and early intervention are important factors for affecting the progression of the disease and improving its prognosis. 展开更多
关键词 hemophagocytic syndrome Epstein-Barr Viru DIAGNOSIS TREATMENT
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Acute Kidney Injury with Epstein Barr Virus-Associated Hemophagocytic Syndrome
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作者 Ryo Ishida Kazumi Komaki +1 位作者 Tetsuro Kusaba Tsuneyuki Nakanouchi 《Case Reports in Clinical Medicine》 2016年第7期233-237,共6页
A 59-year-old man, who was treated for schizophrenia and psoriasis vulgaris with risperidone and cyclosporine, presented with high fever and myalgia. Those did not respond to treatment for neuroleptic malignant syndro... A 59-year-old man, who was treated for schizophrenia and psoriasis vulgaris with risperidone and cyclosporine, presented with high fever and myalgia. Those did not respond to treatment for neuroleptic malignant syndrome for two weeks, and multiple organ dysfunction developed, so he was admitted to our hospital, but died two days later. Autopsy detected the hemophagocytosis, Epstein Barr Virus (EBV)-reactivated cells, and the absence of glomerulonephritis and interstitial tubulitis. We considered that hemophagocytic syndrome (HPS) and myalgia were caused by reactivated EBV and the viremia under immunosuppression, and renal failure was caused by sepsis-like state by cytokine storm of HPS. 展开更多
关键词 hemophagocytic syndrome Epstein Barr Virus Acute Kidney Injury
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Successful treatment of adult-onset still disease caused by pulmonary infection-associated hemophagocytic lymphohistiocytosis: A case report 被引量:1
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作者 Gui Wang Xiao-Rong Jin De-Xun Jiang 《World Journal of Clinical Cases》 SCIE 2020年第3期560-567,共8页
BACKGROUND Adult-onset still disease(AOSD) and hemophagocytic syndrome(HPS) are two inflammatory diseases with very similar clinical manifestations. HPS is one of the most serious complications of AOSD and its risk of... BACKGROUND Adult-onset still disease(AOSD) and hemophagocytic syndrome(HPS) are two inflammatory diseases with very similar clinical manifestations. HPS is one of the most serious complications of AOSD and its risk of death is very high. It is difficult to identify HPS early in patients with AOSD, but early identification and proper treatment directly affects the prognosis.CASE SUMMARY A 39-year-old male showed a high spiking fever and myalgia. Laboratory data revealed elevated white blood cell, serum ferritin, and neutrophil percentage.However, his fever failed to relieve after a clear diagnosis of AOSD caused by pulmonary infection and treatment by antibiotics and corticosteroids;further laboratory data showed elevated serum ferritin, C-reactive protein, erythrocyte sedimentation rate and triglyceride, as well as liver abnormalities. Bone marrow smear showed hemophagocytosis. Secondary HPS was definitely diagnosed. The high fever disappeared and the laboratory findings returned to normal values after treatment by high-dose intravenous methylprednisolone and methotrexate.CONCLUSION For AOSD patients with high suspicion of HPS, active examination needs to be considered for early diagnosis, and timely using of adequate amount of corticosteroids is the key to reducing risk of HPS death. 展开更多
关键词 hemophagocytic syndrome Adult-onset still disease hemophagocytOSIS Case report
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<i>γδ</i>-T Large Granular Lymphocyte Leukemia Associated Hemaphagocytic Syndrome Complicated with Multiple Organ Dysfunction
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作者 Suigui Wan Chengcheng Zheng +3 位作者 Yang Lin Hong Zhao Li Su Changqing Xia 《Case Reports in Clinical Medicine》 2014年第4期211-215,共5页
Hemophagocytic syndrome (HPS) usually presents as a complication of T/NK cell lymphoma. However, γδ-T large granular lymphocyte leukemia (LGL) associated HPS was rarely reported. Herein, we reported a case of γδ-T... Hemophagocytic syndrome (HPS) usually presents as a complication of T/NK cell lymphoma. However, γδ-T large granular lymphocyte leukemia (LGL) associated HPS was rarely reported. Herein, we reported a case of γδ-T LGL associated HPS. A previously healthy 21-year-old Chinese man was admitted with high fever, severe pancytopenia and liver dysfunction. He developed disseminated intravascular coaggulation (DIC), shock, gastrointestinal bleeding, acute renal failure as well as central nervous system involvement. Bone marrow aspiration showed infiltration of large granular lymphocytes and hemophagocytic histiocytes. Immunophenotyping analysis by four-color flow cytometry showed the leukemic cells with large side scatter (SSC), CD3+, CD2+, CD8+, CD5-, CD7 partly+, CD4-, CD56-, CD57-. Polymerase chain reaction (PCR) amplification of TCRβ and γ chain gene rearrangement confirmed TCRγδ T cell clone. Thus, a diagnosis of γδ-T LGL associated HPS was established. The patient worsened rapidly and died of multiple organ failure. 展开更多
关键词 γδ-T Large Granular LYMPHOCYTE LEUKEMIA hemophagocytic syndrome Multiple Organ Failure
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Detection of Th17/Treg balance in peripheral blood of children with hemophilia syndrome and its correlation with severity
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作者 Wei-Juan Lei Jie Pan 《Journal of Hainan Medical University》 2019年第5期58-60,共3页
Objective:To investigate the relationship between Th17/Treg balance in peripheral blood and severity of hemophagocytic syndrome in children.Methods:A total of 60 children with HLH diagnosed in our hospital were taken ... Objective:To investigate the relationship between Th17/Treg balance in peripheral blood and severity of hemophagocytic syndrome in children.Methods:A total of 60 children with HLH diagnosed in our hospital were taken as HLH group during January 2015 to October 2018, 100 cases of healthy children underwent physical examination in our hospital were taken as Normal control group. Levels of Th17/Treg balance, ferritin (SF) and inflammatory mediators in peripheral blood were compared between two groups.Results: Ratio of Th17 cells and Th17/Treg ratio in peripheral blood of HLH group were higher than those of Normal control group, and ratio of Treg cells was lower than that of Normal control group. Levels of serum SF in HLH group were lower than those in Normal control group, and levels of inflammatory mediators such as PCT, IL-6, IL-10 and IFN-γ in peripheral blood were significantly higher than those in Normal control group. Correlation analysis showed that proportion of Th17 cells and the ratio of Th17/Treg in peripheral blood of children with HLH were positively correlated with the contents of SF, PCT, IL-6, IL-10 and IFN-γ, while proportion of Treg cells was negatively correlated with the contents of SF, PCT, IL-6, IL-10 and IFN-γ.Conclusion: Th17/Treg imbalance exists in children with HLH, and there is a positive correlation between Th17/Treg imbalance and the severity of the disease, which may have a certain clinical significance in disease evaluation. 展开更多
关键词 hemophagocytic syndrome TH17/TREG SF Inflammatory MEDIATORS
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以“肾占位”起病的类鼻疽脓毒症合并噬血细胞综合征1例
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作者 黄松 陈强 +1 位作者 曾宇 陈朝彦 《中国感染控制杂志》 CAS CSCD 北大核心 2024年第2期238-241,共4页
类鼻疽病是由类鼻疽伯克霍尔德菌感染所致的传染性疾病,其临床表现多样,常反复发作及合并脓毒症,且易误诊误治,故病死率较高。本文分析1例类鼻疽脓毒症合并噬血细胞综合征的病例,并结合国内外文献对该病的临床特点、诊治思路等进一步总... 类鼻疽病是由类鼻疽伯克霍尔德菌感染所致的传染性疾病,其临床表现多样,常反复发作及合并脓毒症,且易误诊误治,故病死率较高。本文分析1例类鼻疽脓毒症合并噬血细胞综合征的病例,并结合国内外文献对该病的临床特点、诊治思路等进一步总结,以期降低该病的误诊误治,提升患者存活率。 展开更多
关键词 类鼻疽 类鼻疽伯克霍尔德菌 脓毒症 噬血细胞综合征
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