The effects of early hemofiltration on the serum levels of cytokines, pro- and anti-inflammatory balance and organ function in pigs with severe acute pancreatits (SAP) were studied. SAP pig model was induced by retrog...The effects of early hemofiltration on the serum levels of cytokines, pro- and anti-inflammatory balance and organ function in pigs with severe acute pancreatits (SAP) were studied. SAP pig model was induced by retrograde injection of artificial bile into the pancreatic duct. The pigs were randomly divided into SAP hemofiltration treatment group (HF group, n=8) and SAP non-hemofiltration treatment group (NHF group, n=8). In the HF group, the animals were subjected to high-volume and zero-balance hemofiltration therapy. The results showed that as compared with NHF group, MAP, CVP and PaO 2/FiO 2 were significantly increased (P<0.01), while HR, urinary protein content, serum ALT level, pulmonary coefficient and lung wet/dry ratio obviously decreased (P<0.05) in HF group. Under a light microscope, the pulmonary histologic scoring was lower that in HF group (P<0.01) and the lesions of renal and liver tissues were milder. However, there was no significant difference in the pancreatic histologic scoring between the two groups. Six h after establishment of the model, the serum levels of TNF-α, IL-1β were lower, while the IL-10/TNF-α ratio was higher in HF group (all P<0.05). It was suggested that early hemofiltration could effectively remove the serum cytokines TNF-α and IL-1β in SAP pigs, elevate the ratio of IL-10/TNF-α, improve hemodynamics and alleviate the lesions of lung, kidney and liver tissues.展开更多
BACKGROUND We report a rare case of full neurological recovery from severe nonexertional heat stroke in a 67-year-old woman with an initial Glasgow Coma Scale of 3.This report raises awareness among doctors that when ...BACKGROUND We report a rare case of full neurological recovery from severe nonexertional heat stroke in a 67-year-old woman with an initial Glasgow Coma Scale of 3.This report raises awareness among doctors that when heatstroke is diagnosed,comprehensive treatment should be implemented as soon as possible.Moreover,targeted temperature management,combination therapy with hemodialysis and hemoperfusion,and hyperbaric oxygen therapy may alleviate multiorgan failure and prevent neurological sequelae caused by heatstroke.CASE SUMMARY A previously healthy 67-year-old woman with an initial Glasgow Coma Scale of 3 was found lying prone on the road at noon on a summer day.Laboratory tests revealed multiorgan failure.As soon as heatstroke was diagnosed,comprehensive treatment was implemented.On hospital Day 3,the patient was extubated.Her initial Sequential Organ Failure Assessment score at hospitalization was 14 and decreased to 2 on hospital Day 4.On the seventh day following hospital admission,as the patient’s general condition improved,the levels of laboratory test findings decreased rapidly.Finally,the patient gradually recovered with no other neurological symptoms(the Glasgow Coma Scale at discharge was 15,and her ability to walk independently was restored).CONCLUSION This case demonstrated that targeted temperature management,combination therapy with hemodialysis and hemoperfusion,and hyperbaric oxygen therapy may alleviate multiorgan failure and prevent neurological sequelae caused by heatstroke.展开更多
BACKGROUND Adult-onset Still’s disease(AOSD)is a rare systemic inflammatory disease,which is characterized by daily fever and arthritis,with an evanescent rash and neutrophilic leukocytosis.To date,there has been no ...BACKGROUND Adult-onset Still’s disease(AOSD)is a rare systemic inflammatory disease,which is characterized by daily fever and arthritis,with an evanescent rash and neutrophilic leukocytosis.To date,there has been no definite laboratory or imaging test available for diagnosing AOSD;the diagnosis is one of exclusion,which can be very challenging.In particular,AOSD patients may experience different complications affecting their clinical picture,management,and prognosis.The treatment of AOSD remains largely empirical and involves therapeutic agents.CASE SUMMARY We report the case of a 36-year-old woman who presented with fever,red rash,arthralgia,and sore throat.Her serum ferritin level and white blood cell count were markedly elevated,and the first diagnosis 22 years prior was"juvenile rheumatoid arthritis of systemic type".The patient was treated with prednisone,sulfasalazine,methotrexate,and leflunomide.After remission of her symptoms,the patient stopped taking the medications,and the disease recurred.Ultimately,the patient was diagnosed with adult-onset Still's disease.Relapse occurred several times due to self-medication withdrawal,and an interleukin-6 antagonist(tocilizumab/Actemra)was administered to control the disease.Recently,she was hospitalized because an incision did not heal,and the patient suddenly developed high fever and diarrhea during hospitalization.The patient's disease progressed violently and quickly developed into macrophage activation syndrome,disseminated intravascular coagulation,shock,and multiple organ failure.The patient had sudden cardiac arrest,and she died despite emergency rescue efforts.CONCLUSION AOSD patients need regular follow-up in the long-term treatment process,and must press formulary standard medication,and do not voluntarily withdraw or reduce the dose.Otherwise it may cause disease back-and-forth or serious lifethreatening complications.Meanwhile,strict management of trauma,infections,tumors,and other diseases may contribute to improved outcomes in patients with complications.展开更多
BACKGROUND Multiple acyl-CoA dehydrogenase deficiency(MADD)is a disease of rare autosomal recessive disorder.There are three types of MADD.Type I is a neonatalonset form with congenital anomalies.Type II is a neonatal...BACKGROUND Multiple acyl-CoA dehydrogenase deficiency(MADD)is a disease of rare autosomal recessive disorder.There are three types of MADD.Type I is a neonatalonset form with congenital anomalies.Type II is a neonatal-onset form without congenital anomalies.Type III is considered to a milder form and usually responds to riboflavin.However,late-onset form could also be fatal and not responsive to treatments.CASE SUMMARY We report a severe case of a young man with onset type III MADD induced by drugs and strenuous exercise characterized by rhabdomyolysis and liver dysfunction.Urine analysis indicated 12 out of 70 kinds of organic acids like glutaric acid-2 were detected.Serum analysis in genetic metabolic diseases revealed 24 out of 43 tested items were abnormal,revealing the elevation of several acylcarnitines and the reduction of carnitine in the patient.By next generation sequencing technology for gene sequencing related to fatty acid oxidation and carnitine cycle defects,a rare ETFDH gene variant was identified:NM_004453:4:C.1448C>T(p.Pro483 Leu).The patient was diagnosed with lateonset GAII.He was not responsive to riboflavin and progressively worsened into multiple organ failure that finally led to death.CONCLUSION Type III MADD can also be fatal and not responsive to treatments.展开更多
Fifty-seven severely burned patients were divided into 2 groups:16 withmultiple organ failure（MOF）,and 41 without MOF.It was found that the levelof thromboxane B<sub>2</sub>（TXB<sub>2</sub>...Fifty-seven severely burned patients were divided into 2 groups:16 withmultiple organ failure（MOF）,and 41 without MOF.It was found that the levelof thromboxane B<sub>2</sub>（TXB<sub>2</sub>）and the ratio between TXB<sub>2</sub> and 6-keto-prostaglandinF<sub>1α</sub>(TBX<sub>2</sub>/6-keto-PGF<sub>1α</sub>)in plasma and in visceral tissues were increased and re-mained significantly high in the first 5～7 d postburn in patients with MOF but notso in those without MOF.The circulatory platelet aggregate ratio（CPAR）wasmarkedly decreased in the same period in MOF group.Myocardial enzymes（CPK,LDH,and GOT）were markedly increased in the first 3d and remainedsignificantly high within 7 d postburn.Degeneration,structural destruction,edema,hemorrhage and thrombosis were revealed in cardiac,pulmonary,renal andhepatic tissues succumbing to functional failure.Thirteen out of the 16 cases de-veloped MOF during the 3rd to 7th day posthurn and 11 died in that period.These findings substantiate that persistent increase of thromboxane andthromboxane/prostacyclin ratio is closely related to the origin and development ofMOF after burn injury.展开更多
AIMS We report 15 cases of abdominal-surgical multiple organ failure(MOF)treated successfully by a new combined therapy four high doses in volume and one support“FHDOS”short period high doses in large volume anJsoda...AIMS We report 15 cases of abdominal-surgical multiple organ failure(MOF)treated successfully by a new combined therapy four high doses in volume and one support“FHDOS”short period high doses in large volume anJsodaminum;short period high doses in large volume dexamethasonum;high doses in large volume of dis- infectant antibiotics;high doses in large volume of abdominal cav- ity washing liquor and support of nutrition metabolism.The mortal- ity rate and cost of MOF were decreased.It is simple,practical and effective. METHODS The study group consisted of 15 patients(10 wom- en,5 men;average age,42.7)who were hospitalized in our hospital.All patients were given“FHDOS”:short period high in large volume Anisodaminum:intravenous injection 40 mg once; another 40rag may be added 30 minutes later according to condition.The total amount may reach 120-240 mg a day,or,in- travenous injection 40 mg every 15 minutes until condition being under control.Short period high doses in large volume Dexamethasonum:intravenous injection 100-200 mg once;this remedy may be continued for 1-3 days;the amount also may de- crease according to condition.High doses in large volume of dis- infectant Antibiotics:select and use antibiotics according to clini- cal condition,inference of Gram's stain;it is necessary to re- peatedly culture bacteria in many parts,and then,to make ad- justment according to culture result and drug-resistance,and to prevent carefully the dual infections from occurring.High doses in large volume of abdominal cavity washing liquor:generally adopt- ing physiological saline 6000o8000ml in washing abdominal cavity. “One support”means full support of nutrition metabolism. RESULTS All the patients in this group survive well after the rescue without any complication. CONCLUSIONS MOF should be prevented,if possible,by stopping or controlling the injury,removing as much necrotic tis- sue as possible,improving blood flow and oxygen consumption, supporting metabolism,and preventing infection or treating it ear- ly and adequately.“FHDOS”is a combined therapy and plays a key role in treating MOF.展开更多
A hypernatremia severity is often associated with a poor prognosis, especially if it is associated with multiple organ failure. In a country with very limited resources, the prognosis may be favorable in the absence o...A hypernatremia severity is often associated with a poor prognosis, especially if it is associated with multiple organ failure. In a country with very limited resources, the prognosis may be favorable in the absence of renal replacement. We report the case of a 63 years old woman, hypertensive and diabetic, admitted to the ICU for unconsciousness. Clinically, neurological examination notes a Glasgow of 8/15 (Y2, V2, M4) and a left hemiplegia. Temperature was 39°C. Diuresis was 100 ml during the first 24 hours. Blood pressure was 90/60 mmHg, tachycardia at 133/min. Cardiac auscultation is normal. Vascular axes were weakly perceptible. Oxygen saturation was 95%. The skin examination notes a dry skin and mucous membranes, a flattening of the superficial veins, sunken eyes and a persistent skin fold. There is no hepatomegaly or splenomegaly, or jaundice. Biological point of view note natremia: 176 mmol/L;osmolarity: 390 mosmol/kg;creatinin: 300 μmol/L;glycemia > 6 g/L;transaminases 217 UI. Diagnostics: malignant hypernatremia with a high plasma osmolarity associated with an acute anuric renal failure, hydro electrolytic disorders, an abnormal liver function, a fever of central origin and a stroke. The treatment consisted of a correction of the electrolyte disorders by infusion of isotonic and hypotonic fluids with insulin. In a country with very limited resources, the severe hypernatremia prognosis associated with anuric acute renal failure may be favorable in the absence of renal replacement.展开更多
Objective:To analyze the clinical and follow-up data of patients with multiple organ failure of melioidosis pneumonia,to strengthen the understanding of melioidosis,and to provide references for the diagnosis and trea...Objective:To analyze the clinical and follow-up data of patients with multiple organ failure of melioidosis pneumonia,to strengthen the understanding of melioidosis,and to provide references for the diagnosis and treatment of clinicians,especially doctors in Hai nan province with high incidence,so as to reduce misdiagnosis,improve the diagnosis rate and reduce the mortality.Methods:The clinical symptoms,signs,laboratory examination and follow-up of patients with multiple organ failure were analyzed retrospectively.Results:Multiple organ failure with melioidosis pneumonia was easily misdiagnosed,Treatment cycle is long,easy to relapse.Conclusion:It is very important to analyze and differentiate the anthrax with multiple organ failure,especially in Hai nan province,which can improve the diagnosis rate and reduce the mortality.展开更多
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
文摘The effects of early hemofiltration on the serum levels of cytokines, pro- and anti-inflammatory balance and organ function in pigs with severe acute pancreatits (SAP) were studied. SAP pig model was induced by retrograde injection of artificial bile into the pancreatic duct. The pigs were randomly divided into SAP hemofiltration treatment group (HF group, n=8) and SAP non-hemofiltration treatment group (NHF group, n=8). In the HF group, the animals were subjected to high-volume and zero-balance hemofiltration therapy. The results showed that as compared with NHF group, MAP, CVP and PaO 2/FiO 2 were significantly increased (P<0.01), while HR, urinary protein content, serum ALT level, pulmonary coefficient and lung wet/dry ratio obviously decreased (P<0.05) in HF group. Under a light microscope, the pulmonary histologic scoring was lower that in HF group (P<0.01) and the lesions of renal and liver tissues were milder. However, there was no significant difference in the pancreatic histologic scoring between the two groups. Six h after establishment of the model, the serum levels of TNF-α, IL-1β were lower, while the IL-10/TNF-α ratio was higher in HF group (all P<0.05). It was suggested that early hemofiltration could effectively remove the serum cytokines TNF-α and IL-1β in SAP pigs, elevate the ratio of IL-10/TNF-α, improve hemodynamics and alleviate the lesions of lung, kidney and liver tissues.
文摘BACKGROUND We report a rare case of full neurological recovery from severe nonexertional heat stroke in a 67-year-old woman with an initial Glasgow Coma Scale of 3.This report raises awareness among doctors that when heatstroke is diagnosed,comprehensive treatment should be implemented as soon as possible.Moreover,targeted temperature management,combination therapy with hemodialysis and hemoperfusion,and hyperbaric oxygen therapy may alleviate multiorgan failure and prevent neurological sequelae caused by heatstroke.CASE SUMMARY A previously healthy 67-year-old woman with an initial Glasgow Coma Scale of 3 was found lying prone on the road at noon on a summer day.Laboratory tests revealed multiorgan failure.As soon as heatstroke was diagnosed,comprehensive treatment was implemented.On hospital Day 3,the patient was extubated.Her initial Sequential Organ Failure Assessment score at hospitalization was 14 and decreased to 2 on hospital Day 4.On the seventh day following hospital admission,as the patient’s general condition improved,the levels of laboratory test findings decreased rapidly.Finally,the patient gradually recovered with no other neurological symptoms(the Glasgow Coma Scale at discharge was 15,and her ability to walk independently was restored).CONCLUSION This case demonstrated that targeted temperature management,combination therapy with hemodialysis and hemoperfusion,and hyperbaric oxygen therapy may alleviate multiorgan failure and prevent neurological sequelae caused by heatstroke.
文摘BACKGROUND Adult-onset Still’s disease(AOSD)is a rare systemic inflammatory disease,which is characterized by daily fever and arthritis,with an evanescent rash and neutrophilic leukocytosis.To date,there has been no definite laboratory or imaging test available for diagnosing AOSD;the diagnosis is one of exclusion,which can be very challenging.In particular,AOSD patients may experience different complications affecting their clinical picture,management,and prognosis.The treatment of AOSD remains largely empirical and involves therapeutic agents.CASE SUMMARY We report the case of a 36-year-old woman who presented with fever,red rash,arthralgia,and sore throat.Her serum ferritin level and white blood cell count were markedly elevated,and the first diagnosis 22 years prior was"juvenile rheumatoid arthritis of systemic type".The patient was treated with prednisone,sulfasalazine,methotrexate,and leflunomide.After remission of her symptoms,the patient stopped taking the medications,and the disease recurred.Ultimately,the patient was diagnosed with adult-onset Still's disease.Relapse occurred several times due to self-medication withdrawal,and an interleukin-6 antagonist(tocilizumab/Actemra)was administered to control the disease.Recently,she was hospitalized because an incision did not heal,and the patient suddenly developed high fever and diarrhea during hospitalization.The patient's disease progressed violently and quickly developed into macrophage activation syndrome,disseminated intravascular coagulation,shock,and multiple organ failure.The patient had sudden cardiac arrest,and she died despite emergency rescue efforts.CONCLUSION AOSD patients need regular follow-up in the long-term treatment process,and must press formulary standard medication,and do not voluntarily withdraw or reduce the dose.Otherwise it may cause disease back-and-forth or serious lifethreatening complications.Meanwhile,strict management of trauma,infections,tumors,and other diseases may contribute to improved outcomes in patients with complications.
文摘BACKGROUND Multiple acyl-CoA dehydrogenase deficiency(MADD)is a disease of rare autosomal recessive disorder.There are three types of MADD.Type I is a neonatalonset form with congenital anomalies.Type II is a neonatal-onset form without congenital anomalies.Type III is considered to a milder form and usually responds to riboflavin.However,late-onset form could also be fatal and not responsive to treatments.CASE SUMMARY We report a severe case of a young man with onset type III MADD induced by drugs and strenuous exercise characterized by rhabdomyolysis and liver dysfunction.Urine analysis indicated 12 out of 70 kinds of organic acids like glutaric acid-2 were detected.Serum analysis in genetic metabolic diseases revealed 24 out of 43 tested items were abnormal,revealing the elevation of several acylcarnitines and the reduction of carnitine in the patient.By next generation sequencing technology for gene sequencing related to fatty acid oxidation and carnitine cycle defects,a rare ETFDH gene variant was identified:NM_004453:4:C.1448C>T(p.Pro483 Leu).The patient was diagnosed with lateonset GAII.He was not responsive to riboflavin and progressively worsened into multiple organ failure that finally led to death.CONCLUSION Type III MADD can also be fatal and not responsive to treatments.
文摘Fifty-seven severely burned patients were divided into 2 groups:16 withmultiple organ failure（MOF）,and 41 without MOF.It was found that the levelof thromboxane B<sub>2</sub>（TXB<sub>2</sub>）and the ratio between TXB<sub>2</sub> and 6-keto-prostaglandinF<sub>1α</sub>(TBX<sub>2</sub>/6-keto-PGF<sub>1α</sub>)in plasma and in visceral tissues were increased and re-mained significantly high in the first 5～7 d postburn in patients with MOF but notso in those without MOF.The circulatory platelet aggregate ratio（CPAR）wasmarkedly decreased in the same period in MOF group.Myocardial enzymes（CPK,LDH,and GOT）were markedly increased in the first 3d and remainedsignificantly high within 7 d postburn.Degeneration,structural destruction,edema,hemorrhage and thrombosis were revealed in cardiac,pulmonary,renal andhepatic tissues succumbing to functional failure.Thirteen out of the 16 cases de-veloped MOF during the 3rd to 7th day posthurn and 11 died in that period.These findings substantiate that persistent increase of thromboxane andthromboxane/prostacyclin ratio is closely related to the origin and development ofMOF after burn injury.
文摘AIMS We report 15 cases of abdominal-surgical multiple organ failure(MOF)treated successfully by a new combined therapy four high doses in volume and one support“FHDOS”short period high doses in large volume anJsodaminum;short period high doses in large volume dexamethasonum;high doses in large volume of dis- infectant antibiotics;high doses in large volume of abdominal cav- ity washing liquor and support of nutrition metabolism.The mortal- ity rate and cost of MOF were decreased.It is simple,practical and effective. METHODS The study group consisted of 15 patients(10 wom- en,5 men;average age,42.7)who were hospitalized in our hospital.All patients were given“FHDOS”:short period high in large volume Anisodaminum:intravenous injection 40 mg once; another 40rag may be added 30 minutes later according to condition.The total amount may reach 120-240 mg a day,or,in- travenous injection 40 mg every 15 minutes until condition being under control.Short period high doses in large volume Dexamethasonum:intravenous injection 100-200 mg once;this remedy may be continued for 1-3 days;the amount also may de- crease according to condition.High doses in large volume of dis- infectant Antibiotics:select and use antibiotics according to clini- cal condition,inference of Gram's stain;it is necessary to re- peatedly culture bacteria in many parts,and then,to make ad- justment according to culture result and drug-resistance,and to prevent carefully the dual infections from occurring.High doses in large volume of abdominal cavity washing liquor:generally adopt- ing physiological saline 6000o8000ml in washing abdominal cavity. “One support”means full support of nutrition metabolism. RESULTS All the patients in this group survive well after the rescue without any complication. CONCLUSIONS MOF should be prevented,if possible,by stopping or controlling the injury,removing as much necrotic tis- sue as possible,improving blood flow and oxygen consumption, supporting metabolism,and preventing infection or treating it ear- ly and adequately.“FHDOS”is a combined therapy and plays a key role in treating MOF.
文摘A hypernatremia severity is often associated with a poor prognosis, especially if it is associated with multiple organ failure. In a country with very limited resources, the prognosis may be favorable in the absence of renal replacement. We report the case of a 63 years old woman, hypertensive and diabetic, admitted to the ICU for unconsciousness. Clinically, neurological examination notes a Glasgow of 8/15 (Y2, V2, M4) and a left hemiplegia. Temperature was 39°C. Diuresis was 100 ml during the first 24 hours. Blood pressure was 90/60 mmHg, tachycardia at 133/min. Cardiac auscultation is normal. Vascular axes were weakly perceptible. Oxygen saturation was 95%. The skin examination notes a dry skin and mucous membranes, a flattening of the superficial veins, sunken eyes and a persistent skin fold. There is no hepatomegaly or splenomegaly, or jaundice. Biological point of view note natremia: 176 mmol/L;osmolarity: 390 mosmol/kg;creatinin: 300 μmol/L;glycemia > 6 g/L;transaminases 217 UI. Diagnostics: malignant hypernatremia with a high plasma osmolarity associated with an acute anuric renal failure, hydro electrolytic disorders, an abnormal liver function, a fever of central origin and a stroke. The treatment consisted of a correction of the electrolyte disorders by infusion of isotonic and hypotonic fluids with insulin. In a country with very limited resources, the severe hypernatremia prognosis associated with anuric acute renal failure may be favorable in the absence of renal replacement.
基金National Natural Science Foundation of China(No.81660010,31660329,8191101552)
文摘Objective:To analyze the clinical and follow-up data of patients with multiple organ failure of melioidosis pneumonia,to strengthen the understanding of melioidosis,and to provide references for the diagnosis and treatment of clinicians,especially doctors in Hai nan province with high incidence,so as to reduce misdiagnosis,improve the diagnosis rate and reduce the mortality.Methods:The clinical symptoms,signs,laboratory examination and follow-up of patients with multiple organ failure were analyzed retrospectively.Results:Multiple organ failure with melioidosis pneumonia was easily misdiagnosed,Treatment cycle is long,easy to relapse.Conclusion:It is very important to analyze and differentiate the anthrax with multiple organ failure,especially in Hai nan province,which can improve the diagnosis rate and reduce the mortality.
文摘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.
