BACKGROUND Hemophagocytic lymphohistiocytosis(HLH) is a rare life-threatening disorder,often resulting in the immune-mediated injury of multiple organ systems,including primary HLH and secondary HLH(sHLH). Among them,...BACKGROUND Hemophagocytic lymphohistiocytosis(HLH) is a rare life-threatening disorder,often resulting in the immune-mediated injury of multiple organ systems,including primary HLH and secondary HLH(sHLH). Among them, sHLH results from infections, malignant, or autoimmune conditions, which have quite poor outcomes even with aggressive management and are more common in adults.CASE SUMMARY We report a rare case of a 36-year-old female manifested with sHLH on background with systemic lupus erythematosus(SLE). During hospitalization, the patient was characterized by recurrent high-grade fever, petechiae and ecchymoses of abdominal skin, and pulmonary infection. Whole exon gene sequencing revealed decreased activity of natural killer cells. She received systematic treatment with Methylprednisolone, Etoposide, and anti-infective drugs. Intravenous immunoglobulin and plasmapheresis were applied when the condition was extremely acute and progressive. The patient recovered and did not present any relapse of the HLH for one year of follow-up.CONCLUSION The case showed sHLH, thrombotic microvascular, and infection in the whole course of the disease, which was rarely reported by now. The treatment of the patient emphasizes that early recognition and treatment of sHLH in SLE patients was of utmost importance to improve the prognosis and survival rate of patients.展开更多
BACKGROUND Angioimmunoblastic T-cell lymphoma(AITL) is a subtype of peripheral T-cell lymphoma, with heterogenous clinical manifestations and poor prognosis. Here,we report a case of AITL induced hemophagocytic lympho...BACKGROUND Angioimmunoblastic T-cell lymphoma(AITL) is a subtype of peripheral T-cell lymphoma, with heterogenous clinical manifestations and poor prognosis. Here,we report a case of AITL induced hemophagocytic lymphohistiocytosis(HLH)and disseminated intravascular coagulopathy(DIC).CASE SUMMARY An 83-year-old man presented with fever and purpura of both lower limbs for one month. Groin lymph node puncture and flow cytometry indicated a diagnosis of AITL. Bone marrow examination and other laboratory related indexes indicated DIC and HLH. The patient rapidly succumbed to gastrointestinal bleeding and septic shock.CONCLUSION This is the first reported case of AITL induced HLH and DIC. AITL is more aggressive in older adults. In addition to male gender, mediastinal lymphadenopathy, anaemia, and sustained high level of neutrophil-to-lymphocyte ratio may indicate a greater risk of death. Early diagnosis, early detection of severe complications, and prompt and effective treatment are vital.展开更多
BACKGROUND Angioimmunoblastic T-cell lymphoma(AITL), a unique subtype of peripheral Tcell lymphoma, has relatively poor outcomes. High-dose chemotherapy with autologous stem cell transplantation(ASCT) can achieve comp...BACKGROUND Angioimmunoblastic T-cell lymphoma(AITL), a unique subtype of peripheral Tcell lymphoma, has relatively poor outcomes. High-dose chemotherapy with autologous stem cell transplantation(ASCT) can achieve complete remission and improve outcomes. Unfortunately, subsequent T-cell lymphoma-triggered hemophagocytic lymphohistiocytosis(HLH) has a worse prognosis than B-cell lymphoma-triggered HLH.CASE SUMMARY We here report a 50-year-old woman with AITL who achieved a favorable outcome after developing HLH 2 mo after receiving high-dose chemotherapy/ASCT. The patient was initially admitted to our hospital because of multiple enlarged lymph nodes. The final pathologic diagnosis, made on biopsy of a left axillary lymph node was AITL(Stage Ⅳ, Group A). Four cycles of the following chemotherapy regimen were administered: Cyclophosphamide 1.3 g, doxorubicin 86 mg, and vincristine 2 mg on day 1;prednisone 100 mg on days 1-5;and lenalidomide 25 mg on days 1-14. The interval between each cycle was 21 d. The patient received a conditioning regimen(busulfan, cyclophosphamide, and etoposide) followed by peripheral blood stem cell infusion. Unfortunately, she developed sustained fever and a low platelet count 17 d after ACST, leading to a diagnosis of HLH after ASCT. During treatment, she experienced thrombocytopenia and Pneumocystis carinii pneumonia. The patient was successfully treated with etoposide and glucocorticoids.CONCLUSION It is possible that development of HLH is related to immune reconstitution after ASCT.展开更多
BACKGROUND Burkholderia pseudomallei(B.pseudomallei)is a short,straight,medium-sized Gramnegative bacterium that mostly exists alone,without a capsule or spores,has more than three flagella at one end,and actively mov...BACKGROUND Burkholderia pseudomallei(B.pseudomallei)is a short,straight,medium-sized Gramnegative bacterium that mostly exists alone,without a capsule or spores,has more than three flagella at one end,and actively moves.B.pseudomallei confers high morbidity and mortality,with frequent granulocytopenia in B.pseudomallei sepsisrelated deaths.However,mortality may be related to hemophagocytic lymphohistiocytosis(HLH)secondary to B.pseudomallei infection.CASE SUMMARY A 12-year-old female was referred from a local hospital to the pediatric intensive care unit with suspected septic shock and fever,cough,dyspnea,and malaise.After admission,supportive symptomatic treatments including fluid resuscitation,anti-infective therapy,mechanical ventilation,and a vasoactive drug maintenance cycle were carefully initiated.The patient became unconscious,her blood pressure could not be maintained even under the exposure of vasoactive drugs,and she experienced cardiorespiratory arrest.The patient died due to ineffective high-quality in-hospital cardiopulmonary resuscitation.A subsequent bone marrow smear examination revealed extensive phagocytosis,and the blood culture was positive for B.pseudomallei.Family history revealed a sibling death from B.pseudomallei sepsis 5 years earlier.CONCLUSION The higher mortality rate in patients with B.pseudomallei sepsis may be related to secondary HLH after infection,wherein multiorgan dysfunction syndrome may be directly related to infection or immune damage caused by secondary HLH.Patients with B.pseudomallei can be asymptomatic and can become an infective source.展开更多
BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare but life-threatening condition.It is an immune-mediated disease that has a wide range of causes,elicits a hyperinflammatory response,and results in multiple ...BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare but life-threatening condition.It is an immune-mediated disease that has a wide range of causes,elicits a hyperinflammatory response,and results in multiple organ damage.Clinical presentations vary,and in some cases,jaundice occurs as the first symptom.CASE SUMMARY We report the case of a 71-year-old female patient who presented with jaundice.She was admitted to our hospital because of the occurrence of“jaundice for half a month”,and upon examination,obstructive jaundice with choledocholithiasis and gallstones was suggested.Cholecystectomy and choledocholithotomy were performed.However,the jaundice did not improve after surgery.We found splenomegaly,cytopenia,hypertriglyceridemia,hypofibrinogenemia,and elevated ferritin.Bone marrow biopsy revealed hemophagocytosis.Later,cardiac arrest occurred when she returned 3 wk after the surgery.We considered that HLH was triggered by septic shock.The patient’s condition deteriorated rapidly,with multiple organ dysfunction and severe gastrointestinal bleeding.Corticosteroid therapy and symptomatic treatment failed to save her life.CONCLUSION Jaundice rarely presents as the first symptom in HLH patients.The HLH in this case was triggered by septic shock with jaundice as the first symptom.Clinicians should try hard to reduce missed diagnoses and misdiagnoses.展开更多
BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a severe hyperinflammatory reaction,which is rare and life-threatening.According to the pathogen,HLH is divided into genetic and acquired.The most common form of ac...BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a severe hyperinflammatory reaction,which is rare and life-threatening.According to the pathogen,HLH is divided into genetic and acquired.The most common form of acquired HLH is infection-associated HLH,of which Herpes viruses,particularly Epstein-Barr virus(EBV),are the leading infectious triggers.However,it is difficult to distinguish between simple infection with EBV and EBV-induced infectionassociated HLH since both can destroy the whole-body system,particularly the liver,thereby increasing the difficulty of diagnosis and treatment.CASE SUMMARY This paper elaborates a case about EBV-induced infection-associated HLH and acute liver injury,aiming to propose clinical guides for the early detection and treatment of patients with EBV-induced infection-associated HLH.The patient was categorized as acquired hemophagocytic syndrome in adults.After the ganciclovir antiviral treatment combined with meropenem antibacterial therapy and methylprednisolone inhibition to inflammatory response,gamma globulin enhanced immunotherapy,the patient recovered.CONCLUSION From the diagnosis and treatment of this patient,attention should be paid to routine EBV detection and a further comprehensive understanding of the disease as well as early recognition and early initiation are keys to patients’survival.展开更多
BACKGROUND Familial hemophagocytic lymphohistiocytosis type 2(FHL2)is a rare genetic disorder presenting with fever,hepatosplenomegaly,and pancytopenia secondary to perforin-1(PRF1)mutation.FLH2 has been described in ...BACKGROUND Familial hemophagocytic lymphohistiocytosis type 2(FHL2)is a rare genetic disorder presenting with fever,hepatosplenomegaly,and pancytopenia secondary to perforin-1(PRF1)mutation.FLH2 has been described in Chinese but usually presents after 1 year old.We describe a female Chinese neonate with FHL2 secondary to compound heterozygous PRF1 mutation with symptom onset before 1 mo old.We review Chinese FHL2 patients in the literature for comparison.CASE SUMMARY A 15-d-old female neonate was referred to our hospital for persistent fever and thrombocytopenia with diffuse petechiae.She was born to a G5P3 mother at 39 wk and 4 d via cesarean section secondary to breech presentation.No resuscitation was required at birth.She was described to be very sleepy with poor appetite since birth.She developed a fever up to 39.5°C at 7 d of life.Leukocytosis,anemia,and thrombocytopenia were detected at a local medical facility CONCLUSION A literature review identified 75 Chinese FHL2 patients,with only five presenting in the first year of life.Missense and frameshift mutations are the most common PRF1 mutations in Chinese,with 24.8%having c.1349C>T followed by 11.6%having c.65delC.The c.658G>C mutation has only been reported once in the literature and our case suggests it can be pathogenic,at least in the presence of another pathogenic mutation such as c.1066C>T.展开更多
BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a life-threatening hyperinflammatory syndrome caused by many genetic defects.STAT1 is a DNAbinding factor that regulates gene transcription.HLH caused by STAT1 gain...BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a life-threatening hyperinflammatory syndrome caused by many genetic defects.STAT1 is a DNAbinding factor that regulates gene transcription.HLH caused by STAT1 gain-offunction(GOF)mutations has rarely been reported and its clinical manifestations and mechanisms are not clearly defined.CASE SUMMARY A 2-year-old boy presented to our hospital with recurrent fever for>20 d.The patient had a personal history of persistent oral candidiasis and inoculation site infection during the past 2 years.Hepatosplenomegaly was noted.Complete blood cell count showed severe anemia,thrombocytopenia and neutropenia.Other laboratory tests showed liver dysfunction,hypertriglyceridemia and decreased fibrinogen.Hemophagocytosis was found in the bone marrow.Chest computed tomography showed a cavitary lesion.Tests for fungal infection were positive.Serum T helper(Th)1/Th2 cytokine determination demonstrated moderately elevated levels of interleukin(IL)-6 and IL-10 with normal interferon(IFN)-γconcentration.Mycobacterium bovis was identified in bronchoalveolar lavage fluid by polymerase chain reaction.Genetic testing identified a heterozygous mutation of c.1154C>T causing a T385M amino acid substitution in STAT1.Despite antibacterial and antifungal therapy,the febrile disease was not controlled.The signs of HLH were relieved after HLH-94 protocol administration,except fever.Fever was not resolved until he received anti-tuberculosis therapy.Hematopoietic stem cell transplantation was refused and the patient died six months later due to severe pneumonia.CONCLUSION Patients with STAT1 GOF mutation have broad clinical manifestations and may develop HLH.This form of HLH presents with normal IFN-γlevel without cytokine storm.展开更多
BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare and life-threatening disease caused by inherited pathogenic mutations and acquired dysregulations of the immune system.Composite lymphoma is defined as two o...BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare and life-threatening disease caused by inherited pathogenic mutations and acquired dysregulations of the immune system.Composite lymphoma is defined as two or more morphologically and immunophenotypically distinct lymphomas that occur in a single patient.Here,we report two cases of HLH secondary to composite lymphoma with mixed lineage features of T-and B-cell marker expression both in the bone marrow and lymph nodes in adult patients.CASE SUMMARY Two patients were diagnosed with HLH based on the occurrence of fever,pancytopenia,lymphadenopathy,splenomegaly,hemophagocytosis and hyperferritinemia.Immunohistochemical staining of the axillary lymph node and bone marrow in case 1 showed typical features of combined B-cell and T-cell lymphoma.In addition,a lymph node gene study revealed rearrangement of the T-cell receptor chain and the immunoglobulin gene.Morphology and immunohistochemistry studies of a lymph node biopsy in case 2 showed typical features of T cell lymphoma,but immunophenotyping by flow cytometry analysis of bone marrow aspirate showed B cell lymphoma involvement.The patients were treated with high-dose methylprednisolone combined with etoposide to control aggressive HLH progression.The patients also received immunochemotherapy with the R-CHOP(rituximab,cyclophosphamide,doxorubicin,vincristine,and prednisone)regimen immediately after diagnosis.Both patients presented with highly aggressive lymphoma,and died of severe infection or uncontrolled HLH.CONCLUSION We present two rare cases with overwhelming hemophagocytosis along with composite T-and B-cell lymphoma,which posed a diagnostic dilemma.HLH caused by composite lymphoma was characterized by poor clinical outcomes.展开更多
BACKGROUND Familial hemophagocytic lymphohistiocytosis(FHL)is a primary immunodeficiency disease caused by gene defects.The onset of FHL in adolescents and adults may lead clinicians to ignore or even misdiagnose the ...BACKGROUND Familial hemophagocytic lymphohistiocytosis(FHL)is a primary immunodeficiency disease caused by gene defects.The onset of FHL in adolescents and adults may lead clinicians to ignore or even misdiagnose the disease.To the best of our knowledge,this is the first report to detail the clinical features of type 2 FHL(FHL2)with compound heterozygous perforin(PRF1)defects involving the c.163C>T mutation,in addition to correlation analysis and a literature review.CASE SUMMARY We report a case of a 27-year-old male patient with FHL2,who was admitted with a persistent fever and pancytopenia.Through next-generation sequencing technology of hemophagocytic lymphohistiocytosis(HLH)-related genes,we found compound heterozygous mutations of PRF1:c.65delC(p.Pro22Argfs*29)(frameshift mutation,paternal)and c.163C>T(p.Arg55Cys)(missense mutation,maternal).Although he did not receive hematopoietic stem cell transplantation,the patient achieved complete remission after receiving HLH-2004 treatment protocol.To date,the patient has stopped taking drugs for 15 mo,is in a stable condition,and is under follow-up observation.CONCLUSION The delayed onset of FHL2 may be related to the PRF1 mutation type,pathogenic variation pattern,triggering factors,and the temperature sensitivity of some PRF1 mutations.For individual,the detailed reason for the delay in the onset of FHL warrants further investigation.展开更多
BACKGROUND Aggressive natural killer cell leukemia(ANKL)is a rare natural killer cell neoplasm characterized by systemic infiltration of Epstein–Barr virus and rapidly progressive clinical course.ANKL can be accompan...BACKGROUND Aggressive natural killer cell leukemia(ANKL)is a rare natural killer cell neoplasm characterized by systemic infiltration of Epstein–Barr virus and rapidly progressive clinical course.ANKL can be accompanied with hemophagocytic lymphohistiocytosis(HLH).Here,we report a case of ANKL with rare skin lesions as an earlier manifestation,accompanied with HLH,and review the literature in terms of etiology,clinical manifestation,diagnosis and treatment.CASE SUMMARY A 30-year-old woman from Northwest China presented with the clinical characteristics of jaundice,fever,erythema,splenomegaly,progressive hemocytopenia,liver failure,quantities of abnormal cells in bone marrow,and associated HLH.The immunophenotypes of abnormal cells were positive for CD2,cCD3,CD7,CD56,CD38 and negative for sCD3,CD8 and CD117.The diagnosis of ANKL complicated with HLH was confirmed.Following the initial diagnosis and supplementary treatment,the patient received chemotherapy with VDLP regimen(vincristine,daunorubicin,L-asparaginase and prednisone).However,the patient had severe adverse reactions and complication such as severe hematochezia,neutropenia,and multiple organ dysfunction syndrome,and died a few days later.CONCLUSION This is the first reported case of ANKL with rare skin lesions as an earlier manifestation and associated with HLH.展开更多
BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare but life-threatening disorder,characterized by a hyperimmune response.The mortality is high despite progress being made in the diagnosis and treatment of the...BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare but life-threatening disorder,characterized by a hyperimmune response.The mortality is high despite progress being made in the diagnosis and treatment of the disease.HLH is traditionally divided into primary(familial or genetic)and secondary(reactive)according to the etiology.Secondary HLH(sHLH),more common in adults,is often associated with underlying conditions including severe infections,malignancies,autoimmune diseases,or other etiologies.CASE SUMMARY The case involves a 31-year-old woman,presented with a high persistent fever,rash,and splenomegaly.She met the diagnostic criteria of the HLH-2004 guideline and thus was diagnosed with HLH,with positive anti-nuclear antibody(ANA)and positive cytomegalovirus(CMV)-DNA.The patient responded well to a combination of immunomodulatory,chemotherapy,and supportive treatments.When her PCR evaluation for CMV turned negative,her serum ferritin also dropped significantly.Her clinical symptoms improved dramatically,and except for ANA,the abnormal laboratory findings associated with HLH returned to normal.Our previous study has shown that the median overall survival of HLH patients is only 6 mo;however,our patient has been cured and has not presented with any relapse of the disease for 6 years.CONCLUSION This case emphasizes that thorough early removal of the CMV infection is significant for the prognosis of this HLH patient.展开更多
BACKGROUND Malaria-associated secondary hemophagocytic lymphohistiocytosis(HLH)is rare.Moreover,the literature on malaria-associated HLH is sparse,and there are no similar cases reported in China.CASE SUMMARY We repor...BACKGROUND Malaria-associated secondary hemophagocytic lymphohistiocytosis(HLH)is rare.Moreover,the literature on malaria-associated HLH is sparse,and there are no similar cases reported in China.CASE SUMMARY We report the case of a 29-year-old woman with unexplained intermittent fever who was admitted to our hospital due to an unclear diagnosis.The patient concealed her history of travel to Nigeria before onset.We made a diagnosis of malaria-associated secondary HLH.The treatment strategy for this patient included treatment of the inciting factor(artemether for 9 d followed by artemisinin for 5 d),the use of immunosuppressants(steroids,intravenous immunoglobulin)and supportive care.The patient was discharged in normal physical condition after 25 d of intensive care.No relapses were documented on follow-up at six months and 1 year.CONCLUSION Early diagnosis of the primary disease along with timely intervention and a multidisciplinary approach can help patients achieve a satisfactory outcome.展开更多
BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare disorder with rapid progression and high mortality.HLH occurs mostly due to infection,malignant tumors,and immune disorders.Among infections that cause HLH,v...BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare disorder with rapid progression and high mortality.HLH occurs mostly due to infection,malignant tumors,and immune disorders.Among infections that cause HLH,viral infections,especially Epstein-Barr virus infections,are common,whereas tuberculosis is rare.Tuberculosis-associated HLH has a wide range of serological and clinical manifestations that are similar to those of systemic lupus erythematosus(SLE).CASE SUMMARY This study describes a case of tuberculosis-associated HLH misdiagnosed as SLE because of antinuclear antibody(ANA),Smith(Sm)antibody and lupus anticoagulant positivity;leukopenia;thrombocytopenia;pleural effusion;decreased C3,quantitatively increased 24 h urinary protein and fever.The patient was initially treated with glucocorticoids,which resulted in peripheral blood cytopenia and symptom recurrence.Then,caseating granulomas and hemophagocytosis were observed in her bone marrow.She was successfully treated with conventional category 1 antituberculous drugs.In addition,we reviewed the literature on tuberculosis-associated HLH documented in Pub Med,including all full-text articles published in English from December 2009 to December 2019,and summarized the key points,including the epidemiology,clinical manifestations,diagnosis,and treatment of tuberculosis-associated HLH and the differences of the present case from previous reports.CONCLUSION Tuberculosis should be considered in patients with fever or respiratory symptoms.Antituberculous drugs are important for treating tuberculosisassociated HLH.展开更多
Introduction: Hemophagocytic lymphohistiocytosis during pregnancy is a rare and severe condition, and timely diagnosis is quite difficult. We present the first case of hemophagocytic lymphohistiocytosis caused by pyog...Introduction: Hemophagocytic lymphohistiocytosis during pregnancy is a rare and severe condition, and timely diagnosis is quite difficult. We present the first case of hemophagocytic lymphohistiocytosis caused by pyogenic liver abscess during pregnancy and discuss the clinical presentation. Case Presentation: A 26-year-old Japanese primigravida at 23 weeks of gestation complained of extremely high fever with a fast heart rate (140 beats per minute). She presented with systemic inflammatory response syndrome (SIRS). Only 2 days later, she died. Autopsy findings proved that this was the first case of hemophagocytic lymphohistiocytosis caused by pyogenic liver abscess during pregnancy. Conclusion: Hemophagocytic lymphohistiocytosis should be considered when patients meet the SIRS criteria, especially whose clinical presentation includes extremely high fever (39°C) and a fast heart rate (greater than 110 bpm) during pregnancy, despite relatively normal laboratory data, because such vital signs may be associated with the onset of hemophagocytic lymphohistiocytosis.展开更多
Hemophagocytic lymphohistiocytosis (HLH), which was first described in 1939 by paediatricians Scott and Robb-Smith, is a life-threatening disease. HLH is characterized as cytokine release syndrome which is caused by e...Hemophagocytic lymphohistiocytosis (HLH), which was first described in 1939 by paediatricians Scott and Robb-Smith, is a life-threatening disease. HLH is characterized as cytokine release syndrome which is caused by excessive but non-malignant activation of macrophages and/or histiocytes in bone marrow and other reticuloendothelial systems. EBV-HLH is the most common type of infection-associated HLH, has a high mortality rate without prompt and effective treatment. A previous study showed that the one-year mortality rate of EBV-HLH patients is 75%. Here we report a case of EBV-associated hemophagocytic syndrome in adult, and the lessons from the treatment process. Through this case, we think that for EBV-related HLH, EBV-DNA should also be monitored in addition to hemophagocytosis-related indicators during treatment. In addition, DEP regimen may not be suitable for patients who have received at least partial response, because impaired immunological functioning may lead to EBV and hemophagocytic re-activity.展开更多
BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a severe and potentially deadly condition associated with extensive inflammation and immune activation.Cytokine adsorption may serve as a supportive treatment that ...BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a severe and potentially deadly condition associated with extensive inflammation and immune activation.Cytokine adsorption may serve as a supportive treatment that can stabilize organ function in affected patients by reducing their circulating cytokines levels.To date,no descriptions of clinical experiences associated with the use of HA330-II column hemoadsorption for the treatment of children affected by HLH have been published.CASE SUMMARY We describe the case of an 11-year-old child with Epstein-Barr virus-associated HLH complicated by liver failure.She underwent HA330-II column hemoadsorption and chemotherapy and exhibited reductions in levels of inflammatory cytokines,including interleukin(IL),IL-6,IL-8,IL-10,and interferon-γ.The patient’s condition and laboratory parameters gradually improved with treatment.CONCLUSION Hemoadsorption may play an important role in cytokine storm elimination in children with HLH combined with liver failure and consequent multiple organ failure.展开更多
A case of hemophagocytic lymphohistiocytosis in the Third Affiliated Hospital of Inner Mongolia Medical University was collected and analyzed on the basis of diagnosis,physical examination and treatment.Misdiagnosis o...A case of hemophagocytic lymphohistiocytosis in the Third Affiliated Hospital of Inner Mongolia Medical University was collected and analyzed on the basis of diagnosis,physical examination and treatment.Misdiagnosis of hemophagocytic syndrome is very common since it is a rare disease.So this paper aims to enhance the doctors’awareness of hemophagocytic syndrome during clinical practice.展开更多
The article discusses a case of severe tsutsugamushi disease complicated with hemophagocytic syndrome in Guangdong Maternal and Child Health Hospital and concludes that blood purification technology has significant th...The article discusses a case of severe tsutsugamushi disease complicated with hemophagocytic syndrome in Guangdong Maternal and Child Health Hospital and concludes that blood purification technology has significant therapeutic effect among children with severe HLH complicated with multiple organ dysfunction.展开更多
BACKGROUND Secondary hemophagocytic lymphohistiocytosis(sHLH)triggered by Salmonella enterica serovar Typhimurium is rare in pediatric patients.There is no consensus on how to treat S.typhimurium-triggered sHLH.CASE S...BACKGROUND Secondary hemophagocytic lymphohistiocytosis(sHLH)triggered by Salmonella enterica serovar Typhimurium is rare in pediatric patients.There is no consensus on how to treat S.typhimurium-triggered sHLH.CASE SUMMARY A 9-year-old boy with intermittent fever for 3 d presented to our hospital with positive results for S.typhimurium,human rhinovirus,and Mycoplasma pneumoniae infections.At the time of admission to our institution,the patient’s T helper 1/T helper 2 cytokine levels were 326 pg/mL for interleukin 6(IL-6),9.1 pg/mL for IL-10,and 246.7 pg/mL for interferon-gamma(IFN-γ),for which the ratio of IL-10 to IFN-γwas 0.04.In this study,the patient received meropenem,linezolid,and cefoperazone/sulbactam in combination with high-dose methylprednisolone therapy(10 mg/kg/d for 3 d)and antishock supportive treatment twice.After careful evaluation,this patient did not receive HLH chemotherapy and recovered well.CONCLUSION S.Typhimurium infection-triggered sHLH patient had a ratio of IL-10 to IFN-γ≤1.33,an IL-10 concentration≤10.0 pg/mL,and/or an IFN-γconcentration≤225 pg/mL at admission.Early antimicrobial and supportive treatment was sufficient,and the HLH-94/2004 protocol was not necessary under these conditions.展开更多
文摘BACKGROUND Hemophagocytic lymphohistiocytosis(HLH) is a rare life-threatening disorder,often resulting in the immune-mediated injury of multiple organ systems,including primary HLH and secondary HLH(sHLH). Among them, sHLH results from infections, malignant, or autoimmune conditions, which have quite poor outcomes even with aggressive management and are more common in adults.CASE SUMMARY We report a rare case of a 36-year-old female manifested with sHLH on background with systemic lupus erythematosus(SLE). During hospitalization, the patient was characterized by recurrent high-grade fever, petechiae and ecchymoses of abdominal skin, and pulmonary infection. Whole exon gene sequencing revealed decreased activity of natural killer cells. She received systematic treatment with Methylprednisolone, Etoposide, and anti-infective drugs. Intravenous immunoglobulin and plasmapheresis were applied when the condition was extremely acute and progressive. The patient recovered and did not present any relapse of the HLH for one year of follow-up.CONCLUSION The case showed sHLH, thrombotic microvascular, and infection in the whole course of the disease, which was rarely reported by now. The treatment of the patient emphasizes that early recognition and treatment of sHLH in SLE patients was of utmost importance to improve the prognosis and survival rate of patients.
基金Supported by the National Natural Science Foundation of China,No. 82160692 and No. 82160037。
文摘BACKGROUND Angioimmunoblastic T-cell lymphoma(AITL) is a subtype of peripheral T-cell lymphoma, with heterogenous clinical manifestations and poor prognosis. Here,we report a case of AITL induced hemophagocytic lymphohistiocytosis(HLH)and disseminated intravascular coagulopathy(DIC).CASE SUMMARY An 83-year-old man presented with fever and purpura of both lower limbs for one month. Groin lymph node puncture and flow cytometry indicated a diagnosis of AITL. Bone marrow examination and other laboratory related indexes indicated DIC and HLH. The patient rapidly succumbed to gastrointestinal bleeding and septic shock.CONCLUSION This is the first reported case of AITL induced HLH and DIC. AITL is more aggressive in older adults. In addition to male gender, mediastinal lymphadenopathy, anaemia, and sustained high level of neutrophil-to-lymphocyte ratio may indicate a greater risk of death. Early diagnosis, early detection of severe complications, and prompt and effective treatment are vital.
基金Supported by the Jinan Clinical Medical Science and Technology Innovation Plan,No.202019141Norman Bethune Foundation-Feifan Iron Supplement Project,No.ffbt-C-2022-010.
文摘BACKGROUND Angioimmunoblastic T-cell lymphoma(AITL), a unique subtype of peripheral Tcell lymphoma, has relatively poor outcomes. High-dose chemotherapy with autologous stem cell transplantation(ASCT) can achieve complete remission and improve outcomes. Unfortunately, subsequent T-cell lymphoma-triggered hemophagocytic lymphohistiocytosis(HLH) has a worse prognosis than B-cell lymphoma-triggered HLH.CASE SUMMARY We here report a 50-year-old woman with AITL who achieved a favorable outcome after developing HLH 2 mo after receiving high-dose chemotherapy/ASCT. The patient was initially admitted to our hospital because of multiple enlarged lymph nodes. The final pathologic diagnosis, made on biopsy of a left axillary lymph node was AITL(Stage Ⅳ, Group A). Four cycles of the following chemotherapy regimen were administered: Cyclophosphamide 1.3 g, doxorubicin 86 mg, and vincristine 2 mg on day 1;prednisone 100 mg on days 1-5;and lenalidomide 25 mg on days 1-14. The interval between each cycle was 21 d. The patient received a conditioning regimen(busulfan, cyclophosphamide, and etoposide) followed by peripheral blood stem cell infusion. Unfortunately, she developed sustained fever and a low platelet count 17 d after ACST, leading to a diagnosis of HLH after ASCT. During treatment, she experienced thrombocytopenia and Pneumocystis carinii pneumonia. The patient was successfully treated with etoposide and glucocorticoids.CONCLUSION It is possible that development of HLH is related to immune reconstitution after ASCT.
文摘BACKGROUND Burkholderia pseudomallei(B.pseudomallei)is a short,straight,medium-sized Gramnegative bacterium that mostly exists alone,without a capsule or spores,has more than three flagella at one end,and actively moves.B.pseudomallei confers high morbidity and mortality,with frequent granulocytopenia in B.pseudomallei sepsisrelated deaths.However,mortality may be related to hemophagocytic lymphohistiocytosis(HLH)secondary to B.pseudomallei infection.CASE SUMMARY A 12-year-old female was referred from a local hospital to the pediatric intensive care unit with suspected septic shock and fever,cough,dyspnea,and malaise.After admission,supportive symptomatic treatments including fluid resuscitation,anti-infective therapy,mechanical ventilation,and a vasoactive drug maintenance cycle were carefully initiated.The patient became unconscious,her blood pressure could not be maintained even under the exposure of vasoactive drugs,and she experienced cardiorespiratory arrest.The patient died due to ineffective high-quality in-hospital cardiopulmonary resuscitation.A subsequent bone marrow smear examination revealed extensive phagocytosis,and the blood culture was positive for B.pseudomallei.Family history revealed a sibling death from B.pseudomallei sepsis 5 years earlier.CONCLUSION The higher mortality rate in patients with B.pseudomallei sepsis may be related to secondary HLH after infection,wherein multiorgan dysfunction syndrome may be directly related to infection or immune damage caused by secondary HLH.Patients with B.pseudomallei can be asymptomatic and can become an infective source.
文摘BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare but life-threatening condition.It is an immune-mediated disease that has a wide range of causes,elicits a hyperinflammatory response,and results in multiple organ damage.Clinical presentations vary,and in some cases,jaundice occurs as the first symptom.CASE SUMMARY We report the case of a 71-year-old female patient who presented with jaundice.She was admitted to our hospital because of the occurrence of“jaundice for half a month”,and upon examination,obstructive jaundice with choledocholithiasis and gallstones was suggested.Cholecystectomy and choledocholithotomy were performed.However,the jaundice did not improve after surgery.We found splenomegaly,cytopenia,hypertriglyceridemia,hypofibrinogenemia,and elevated ferritin.Bone marrow biopsy revealed hemophagocytosis.Later,cardiac arrest occurred when she returned 3 wk after the surgery.We considered that HLH was triggered by septic shock.The patient’s condition deteriorated rapidly,with multiple organ dysfunction and severe gastrointestinal bleeding.Corticosteroid therapy and symptomatic treatment failed to save her life.CONCLUSION Jaundice rarely presents as the first symptom in HLH patients.The HLH in this case was triggered by septic shock with jaundice as the first symptom.Clinicians should try hard to reduce missed diagnoses and misdiagnoses.
基金Supported by the National Natural Science Foundation of China,No.82174189Talents Training Program of Seventh People’s Hospital of Shanghai University of Traditional Chinese Medicine,No.JCR2022-01+3 种基金TCM Specialist Disease Alliance Construction Project of Shanghai Municipal Health Commission,No.ZY(2021-2023)-0302Talent Training Project of Senior Master of Chinese Medicine of Shanghai Pudong,No.PDZY-2022-0601Project of Introducing Senior Talents of Chinese Medicine of Shanghai Pudong,No.PDZY-2022-0701Talents Training Program of the Seventh People’s Hospital,Shanghai University of Traditional Chinese Medicine,No.QMX2021-04.
文摘BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a severe hyperinflammatory reaction,which is rare and life-threatening.According to the pathogen,HLH is divided into genetic and acquired.The most common form of acquired HLH is infection-associated HLH,of which Herpes viruses,particularly Epstein-Barr virus(EBV),are the leading infectious triggers.However,it is difficult to distinguish between simple infection with EBV and EBV-induced infectionassociated HLH since both can destroy the whole-body system,particularly the liver,thereby increasing the difficulty of diagnosis and treatment.CASE SUMMARY This paper elaborates a case about EBV-induced infection-associated HLH and acute liver injury,aiming to propose clinical guides for the early detection and treatment of patients with EBV-induced infection-associated HLH.The patient was categorized as acquired hemophagocytic syndrome in adults.After the ganciclovir antiviral treatment combined with meropenem antibacterial therapy and methylprednisolone inhibition to inflammatory response,gamma globulin enhanced immunotherapy,the patient recovered.CONCLUSION From the diagnosis and treatment of this patient,attention should be paid to routine EBV detection and a further comprehensive understanding of the disease as well as early recognition and early initiation are keys to patients’survival.
文摘BACKGROUND Familial hemophagocytic lymphohistiocytosis type 2(FHL2)is a rare genetic disorder presenting with fever,hepatosplenomegaly,and pancytopenia secondary to perforin-1(PRF1)mutation.FLH2 has been described in Chinese but usually presents after 1 year old.We describe a female Chinese neonate with FHL2 secondary to compound heterozygous PRF1 mutation with symptom onset before 1 mo old.We review Chinese FHL2 patients in the literature for comparison.CASE SUMMARY A 15-d-old female neonate was referred to our hospital for persistent fever and thrombocytopenia with diffuse petechiae.She was born to a G5P3 mother at 39 wk and 4 d via cesarean section secondary to breech presentation.No resuscitation was required at birth.She was described to be very sleepy with poor appetite since birth.She developed a fever up to 39.5°C at 7 d of life.Leukocytosis,anemia,and thrombocytopenia were detected at a local medical facility CONCLUSION A literature review identified 75 Chinese FHL2 patients,with only five presenting in the first year of life.Missense and frameshift mutations are the most common PRF1 mutations in Chinese,with 24.8%having c.1349C>T followed by 11.6%having c.65delC.The c.658G>C mutation has only been reported once in the literature and our case suggests it can be pathogenic,at least in the presence of another pathogenic mutation such as c.1066C>T.
文摘BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a life-threatening hyperinflammatory syndrome caused by many genetic defects.STAT1 is a DNAbinding factor that regulates gene transcription.HLH caused by STAT1 gain-offunction(GOF)mutations has rarely been reported and its clinical manifestations and mechanisms are not clearly defined.CASE SUMMARY A 2-year-old boy presented to our hospital with recurrent fever for>20 d.The patient had a personal history of persistent oral candidiasis and inoculation site infection during the past 2 years.Hepatosplenomegaly was noted.Complete blood cell count showed severe anemia,thrombocytopenia and neutropenia.Other laboratory tests showed liver dysfunction,hypertriglyceridemia and decreased fibrinogen.Hemophagocytosis was found in the bone marrow.Chest computed tomography showed a cavitary lesion.Tests for fungal infection were positive.Serum T helper(Th)1/Th2 cytokine determination demonstrated moderately elevated levels of interleukin(IL)-6 and IL-10 with normal interferon(IFN)-γconcentration.Mycobacterium bovis was identified in bronchoalveolar lavage fluid by polymerase chain reaction.Genetic testing identified a heterozygous mutation of c.1154C>T causing a T385M amino acid substitution in STAT1.Despite antibacterial and antifungal therapy,the febrile disease was not controlled.The signs of HLH were relieved after HLH-94 protocol administration,except fever.Fever was not resolved until he received anti-tuberculosis therapy.Hematopoietic stem cell transplantation was refused and the patient died six months later due to severe pneumonia.CONCLUSION Patients with STAT1 GOF mutation have broad clinical manifestations and may develop HLH.This form of HLH presents with normal IFN-γlevel without cytokine storm.
文摘BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare and life-threatening disease caused by inherited pathogenic mutations and acquired dysregulations of the immune system.Composite lymphoma is defined as two or more morphologically and immunophenotypically distinct lymphomas that occur in a single patient.Here,we report two cases of HLH secondary to composite lymphoma with mixed lineage features of T-and B-cell marker expression both in the bone marrow and lymph nodes in adult patients.CASE SUMMARY Two patients were diagnosed with HLH based on the occurrence of fever,pancytopenia,lymphadenopathy,splenomegaly,hemophagocytosis and hyperferritinemia.Immunohistochemical staining of the axillary lymph node and bone marrow in case 1 showed typical features of combined B-cell and T-cell lymphoma.In addition,a lymph node gene study revealed rearrangement of the T-cell receptor chain and the immunoglobulin gene.Morphology and immunohistochemistry studies of a lymph node biopsy in case 2 showed typical features of T cell lymphoma,but immunophenotyping by flow cytometry analysis of bone marrow aspirate showed B cell lymphoma involvement.The patients were treated with high-dose methylprednisolone combined with etoposide to control aggressive HLH progression.The patients also received immunochemotherapy with the R-CHOP(rituximab,cyclophosphamide,doxorubicin,vincristine,and prednisone)regimen immediately after diagnosis.Both patients presented with highly aggressive lymphoma,and died of severe infection or uncontrolled HLH.CONCLUSION We present two rare cases with overwhelming hemophagocytosis along with composite T-and B-cell lymphoma,which posed a diagnostic dilemma.HLH caused by composite lymphoma was characterized by poor clinical outcomes.
基金the Project of Key Talents of Strengthening Health through Science and Education of Yangzhou City,China,No.ZDRC201813.
文摘BACKGROUND Familial hemophagocytic lymphohistiocytosis(FHL)is a primary immunodeficiency disease caused by gene defects.The onset of FHL in adolescents and adults may lead clinicians to ignore or even misdiagnose the disease.To the best of our knowledge,this is the first report to detail the clinical features of type 2 FHL(FHL2)with compound heterozygous perforin(PRF1)defects involving the c.163C>T mutation,in addition to correlation analysis and a literature review.CASE SUMMARY We report a case of a 27-year-old male patient with FHL2,who was admitted with a persistent fever and pancytopenia.Through next-generation sequencing technology of hemophagocytic lymphohistiocytosis(HLH)-related genes,we found compound heterozygous mutations of PRF1:c.65delC(p.Pro22Argfs*29)(frameshift mutation,paternal)and c.163C>T(p.Arg55Cys)(missense mutation,maternal).Although he did not receive hematopoietic stem cell transplantation,the patient achieved complete remission after receiving HLH-2004 treatment protocol.To date,the patient has stopped taking drugs for 15 mo,is in a stable condition,and is under follow-up observation.CONCLUSION The delayed onset of FHL2 may be related to the PRF1 mutation type,pathogenic variation pattern,triggering factors,and the temperature sensitivity of some PRF1 mutations.For individual,the detailed reason for the delay in the onset of FHL warrants further investigation.
文摘BACKGROUND Aggressive natural killer cell leukemia(ANKL)is a rare natural killer cell neoplasm characterized by systemic infiltration of Epstein–Barr virus and rapidly progressive clinical course.ANKL can be accompanied with hemophagocytic lymphohistiocytosis(HLH).Here,we report a case of ANKL with rare skin lesions as an earlier manifestation,accompanied with HLH,and review the literature in terms of etiology,clinical manifestation,diagnosis and treatment.CASE SUMMARY A 30-year-old woman from Northwest China presented with the clinical characteristics of jaundice,fever,erythema,splenomegaly,progressive hemocytopenia,liver failure,quantities of abnormal cells in bone marrow,and associated HLH.The immunophenotypes of abnormal cells were positive for CD2,cCD3,CD7,CD56,CD38 and negative for sCD3,CD8 and CD117.The diagnosis of ANKL complicated with HLH was confirmed.Following the initial diagnosis and supplementary treatment,the patient received chemotherapy with VDLP regimen(vincristine,daunorubicin,L-asparaginase and prednisone).However,the patient had severe adverse reactions and complication such as severe hematochezia,neutropenia,and multiple organ dysfunction syndrome,and died a few days later.CONCLUSION This is the first reported case of ANKL with rare skin lesions as an earlier manifestation and associated with HLH.
基金Natural Science Foundation of Jiangsu Province of China,No.BK20181492the National Key Clinical Department of Laboratory Medicine of China in Nanjing,Key laboratory for Laboratory Medicine of Jiangsu Province,No.ZDXKB2016005。
文摘BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare but life-threatening disorder,characterized by a hyperimmune response.The mortality is high despite progress being made in the diagnosis and treatment of the disease.HLH is traditionally divided into primary(familial or genetic)and secondary(reactive)according to the etiology.Secondary HLH(sHLH),more common in adults,is often associated with underlying conditions including severe infections,malignancies,autoimmune diseases,or other etiologies.CASE SUMMARY The case involves a 31-year-old woman,presented with a high persistent fever,rash,and splenomegaly.She met the diagnostic criteria of the HLH-2004 guideline and thus was diagnosed with HLH,with positive anti-nuclear antibody(ANA)and positive cytomegalovirus(CMV)-DNA.The patient responded well to a combination of immunomodulatory,chemotherapy,and supportive treatments.When her PCR evaluation for CMV turned negative,her serum ferritin also dropped significantly.Her clinical symptoms improved dramatically,and except for ANA,the abnormal laboratory findings associated with HLH returned to normal.Our previous study has shown that the median overall survival of HLH patients is only 6 mo;however,our patient has been cured and has not presented with any relapse of the disease for 6 years.CONCLUSION This case emphasizes that thorough early removal of the CMV infection is significant for the prognosis of this HLH patient.
文摘BACKGROUND Malaria-associated secondary hemophagocytic lymphohistiocytosis(HLH)is rare.Moreover,the literature on malaria-associated HLH is sparse,and there are no similar cases reported in China.CASE SUMMARY We report the case of a 29-year-old woman with unexplained intermittent fever who was admitted to our hospital due to an unclear diagnosis.The patient concealed her history of travel to Nigeria before onset.We made a diagnosis of malaria-associated secondary HLH.The treatment strategy for this patient included treatment of the inciting factor(artemether for 9 d followed by artemisinin for 5 d),the use of immunosuppressants(steroids,intravenous immunoglobulin)and supportive care.The patient was discharged in normal physical condition after 25 d of intensive care.No relapses were documented on follow-up at six months and 1 year.CONCLUSION Early diagnosis of the primary disease along with timely intervention and a multidisciplinary approach can help patients achieve a satisfactory outcome.
文摘BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare disorder with rapid progression and high mortality.HLH occurs mostly due to infection,malignant tumors,and immune disorders.Among infections that cause HLH,viral infections,especially Epstein-Barr virus infections,are common,whereas tuberculosis is rare.Tuberculosis-associated HLH has a wide range of serological and clinical manifestations that are similar to those of systemic lupus erythematosus(SLE).CASE SUMMARY This study describes a case of tuberculosis-associated HLH misdiagnosed as SLE because of antinuclear antibody(ANA),Smith(Sm)antibody and lupus anticoagulant positivity;leukopenia;thrombocytopenia;pleural effusion;decreased C3,quantitatively increased 24 h urinary protein and fever.The patient was initially treated with glucocorticoids,which resulted in peripheral blood cytopenia and symptom recurrence.Then,caseating granulomas and hemophagocytosis were observed in her bone marrow.She was successfully treated with conventional category 1 antituberculous drugs.In addition,we reviewed the literature on tuberculosis-associated HLH documented in Pub Med,including all full-text articles published in English from December 2009 to December 2019,and summarized the key points,including the epidemiology,clinical manifestations,diagnosis,and treatment of tuberculosis-associated HLH and the differences of the present case from previous reports.CONCLUSION Tuberculosis should be considered in patients with fever or respiratory symptoms.Antituberculous drugs are important for treating tuberculosisassociated HLH.
文摘Introduction: Hemophagocytic lymphohistiocytosis during pregnancy is a rare and severe condition, and timely diagnosis is quite difficult. We present the first case of hemophagocytic lymphohistiocytosis caused by pyogenic liver abscess during pregnancy and discuss the clinical presentation. Case Presentation: A 26-year-old Japanese primigravida at 23 weeks of gestation complained of extremely high fever with a fast heart rate (140 beats per minute). She presented with systemic inflammatory response syndrome (SIRS). Only 2 days later, she died. Autopsy findings proved that this was the first case of hemophagocytic lymphohistiocytosis caused by pyogenic liver abscess during pregnancy. Conclusion: Hemophagocytic lymphohistiocytosis should be considered when patients meet the SIRS criteria, especially whose clinical presentation includes extremely high fever (39°C) and a fast heart rate (greater than 110 bpm) during pregnancy, despite relatively normal laboratory data, because such vital signs may be associated with the onset of hemophagocytic lymphohistiocytosis.
文摘Hemophagocytic lymphohistiocytosis (HLH), which was first described in 1939 by paediatricians Scott and Robb-Smith, is a life-threatening disease. HLH is characterized as cytokine release syndrome which is caused by excessive but non-malignant activation of macrophages and/or histiocytes in bone marrow and other reticuloendothelial systems. EBV-HLH is the most common type of infection-associated HLH, has a high mortality rate without prompt and effective treatment. A previous study showed that the one-year mortality rate of EBV-HLH patients is 75%. Here we report a case of EBV-associated hemophagocytic syndrome in adult, and the lessons from the treatment process. Through this case, we think that for EBV-related HLH, EBV-DNA should also be monitored in addition to hemophagocytosis-related indicators during treatment. In addition, DEP regimen may not be suitable for patients who have received at least partial response, because impaired immunological functioning may lead to EBV and hemophagocytic re-activity.
文摘BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a severe and potentially deadly condition associated with extensive inflammation and immune activation.Cytokine adsorption may serve as a supportive treatment that can stabilize organ function in affected patients by reducing their circulating cytokines levels.To date,no descriptions of clinical experiences associated with the use of HA330-II column hemoadsorption for the treatment of children affected by HLH have been published.CASE SUMMARY We describe the case of an 11-year-old child with Epstein-Barr virus-associated HLH complicated by liver failure.She underwent HA330-II column hemoadsorption and chemotherapy and exhibited reductions in levels of inflammatory cytokines,including interleukin(IL),IL-6,IL-8,IL-10,and interferon-γ.The patient’s condition and laboratory parameters gradually improved with treatment.CONCLUSION Hemoadsorption may play an important role in cytokine storm elimination in children with HLH combined with liver failure and consequent multiple organ failure.
文摘A case of hemophagocytic lymphohistiocytosis in the Third Affiliated Hospital of Inner Mongolia Medical University was collected and analyzed on the basis of diagnosis,physical examination and treatment.Misdiagnosis of hemophagocytic syndrome is very common since it is a rare disease.So this paper aims to enhance the doctors’awareness of hemophagocytic syndrome during clinical practice.
文摘The article discusses a case of severe tsutsugamushi disease complicated with hemophagocytic syndrome in Guangdong Maternal and Child Health Hospital and concludes that blood purification technology has significant therapeutic effect among children with severe HLH complicated with multiple organ dysfunction.
基金Supported by Zhejiang Province Health and Wellness Science and Technology Program in 2022,China,No.2022RC202.
文摘BACKGROUND Secondary hemophagocytic lymphohistiocytosis(sHLH)triggered by Salmonella enterica serovar Typhimurium is rare in pediatric patients.There is no consensus on how to treat S.typhimurium-triggered sHLH.CASE SUMMARY A 9-year-old boy with intermittent fever for 3 d presented to our hospital with positive results for S.typhimurium,human rhinovirus,and Mycoplasma pneumoniae infections.At the time of admission to our institution,the patient’s T helper 1/T helper 2 cytokine levels were 326 pg/mL for interleukin 6(IL-6),9.1 pg/mL for IL-10,and 246.7 pg/mL for interferon-gamma(IFN-γ),for which the ratio of IL-10 to IFN-γwas 0.04.In this study,the patient received meropenem,linezolid,and cefoperazone/sulbactam in combination with high-dose methylprednisolone therapy(10 mg/kg/d for 3 d)and antishock supportive treatment twice.After careful evaluation,this patient did not receive HLH chemotherapy and recovered well.CONCLUSION S.Typhimurium infection-triggered sHLH patient had a ratio of IL-10 to IFN-γ≤1.33,an IL-10 concentration≤10.0 pg/mL,and/or an IFN-γconcentration≤225 pg/mL at admission.Early antimicrobial and supportive treatment was sufficient,and the HLH-94/2004 protocol was not necessary under these conditions.
