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
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
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
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
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
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
<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
<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
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
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
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
文摘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.
基金Supported by the Association Foundation Program of Yunnan Science and Technology Department and Kunming Medical University,No.2019FE001-103Yunnan Health Training Project of High Level Talents,No.D-2017053+2 种基金Top Young Experts Training Project for the Academy and Technology in Kunming and Yunnan Province,No.202005AC160066Postdoctoral Training Program of Yunnan Province,No.Ynbh19035Natural Science Foundation of Yunnan Province,No.2019-1-C-25318000002240.
文摘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.
文摘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.
文摘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.
基金Supported by the National Natural Science Foundation of China,No.81774336(to Pang ZH).
文摘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.
文摘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.
文摘<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.
文摘<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.
文摘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.
文摘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.
文摘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.
