BACKGROUND Hypereosinophilic syndrome(HES)is classified as primary,secondary or idiopathic.Idiopathic HES(IHES)has a variable clinical presentation and may involve multiple organs causing severe damage.Hepatic sinusoi...BACKGROUND Hypereosinophilic syndrome(HES)is classified as primary,secondary or idiopathic.Idiopathic HES(IHES)has a variable clinical presentation and may involve multiple organs causing severe damage.Hepatic sinusoidal obstruction syndrome(HSOS)is characterized by damage to the endothelial cells of the hepatic sinusoids of the hepatic venules,with occlusion of the hepatic venules,and hepatocyte necrosis.We report a case of IHES with HSOS of uncertain etiology.CASE SUMMARY A 70-year-old male patient was admitted to our hospital with pruritus and a rash on the extremities for>5 mo.He had previously undergone antiallergic treatment and herbal therapy in the local hospital,but the symptoms recurred.Relevant examinations were completed after admission.Bone marrow aspiration biopsy showed a significantly higher percentage of eosinophils(23%)with approximately normal morphology.Ultrasound-guided hepatic aspiration biopsy indicated HSOS.Contrast-enhanced computed tomography(CT)of the upper abdomen showed hepatic venule congestion with hydrothorax and ascites.The patient was initially diagnosed with IHES and hepatic venule occlusion.Prednisone,low molecular weight heparin and ursodeoxycholic acid were given for treatment,followed by discontinuation of low molecular weight heparin due to ecchymosis.Routine blood tests,biochemical tests,and imaging such as enhanced CT of the upper abdomen and pelvis were reviewed regularly.CONCLUSION Hypereosinophilia may play a facilitating role in the occurrence and development of HSOS.展开更多
BACKGROUND Simultaneous cerebral and myocardial infarction with arterial involvement has not been reported in hypereosinophilic syndrome(HES).Here,we report a patient with HES that was also associated with acute ische...BACKGROUND Simultaneous cerebral and myocardial infarction with arterial involvement has not been reported in hypereosinophilic syndrome(HES).Here,we report a patient with HES that was also associated with acute ischemic stroke,myocardial infarction,and arterial involvement of the left common carotid artery,vertebral arteries,posterior cerebral artery,and coronary artery.CASE SUMMARY A 64-year-old male patient was admitted with headache and right lower extremity weakness.Laboratory tests indicated eosinophilia.Brain magnetic resonance imaging(MRI)showed bilateral and multiple acute infarcts in the border zones.Electrocardiography revealed that T wave was inverted and that the concentration of troponin I was significantly elevated above normal levels.Cardiac echocardiography showed an ejection fraction of 69%with mitral and tricuspid mild regurgitation.Computed tomography angiography detected multiple and localized instances of mild stenosis in the left common carotid artery bifurcation,bilateral vertebral arteries(V5 segment),and the posterior cerebral artery(P2 segment).These were observed together with multiple non-calcified and mixed plaques as well as luminal stenosis in the left circumflex artery,left anterior descending artery,and right coronary artery.The patient was treated with oral methylprednisolone and clopidogrel,after which the absolute eosinophil count fell rapidly to a normal level.After one month,a second brain MRI showed a partial reduction in the size and number of the lesions.CONCLUSION HES can masquerade as ischemic stroke,myocardial infarction,and arterial vascular involvement.The patient reported here recovered very quickly when his eosinophil blood count returned to normal.Early diagnosis and rapid reduction of eosinophils may lead to a good prognosis.展开更多
BACKGROUND Hypereosinophilic syndrome(HES)is a condition characterized by increased eosinophil proliferation in the bone marrow,as well as tissue eosinophilia,often causing organ damage.The cause of the disease is unk...BACKGROUND Hypereosinophilic syndrome(HES)is a condition characterized by increased eosinophil proliferation in the bone marrow,as well as tissue eosinophilia,often causing organ damage.The cause of the disease is unknown.Initial symptoms include fatigue,cough,shortness of breath,myalgia,angioedema,fever,and pneumonia.In addition to the respiratory symptoms,damage to the central nervous system can lead to severe seizures.Here,we report a case with pneumonia and complex partial seizures secondary to HES.CASE SUMMARY A 94-year-old woman was admitted to our hospital for heart failure and bloody stools.After admission,she also showed symptoms of pneumonia.Non-contrast computed tomography of the chest showed pleural effusion and infiltrative shadows.Lower gastrointestinal endoscopy showed multiple ulcers in the sigmoid colon.Blood analyses showed marked eosinophilia(eosinophils 1760/mm3,total leukocytes 6850/mm3).Initial treatment with furosemide 20 mg/d and prednisolone 25 mg/d relieved these symptoms.However,the patient subsequently experienced localised epileptic seizures characterized by bilateral eyelid twitching and eyes rolling upwards,without generalized convulsions,and respiratory arrest occurred.Electroencephalography showed spikes and waves.Non-contrast magnetic resonance imaging of the brain showed extensive periventricular hyperintensity.With administration of levetiracetam 1000 mg/d the epileptic seizures disappeared.However,the patient’s consciousness remained impaired,and her pneumonia worsened again.Two weeks later,she died of pneumonia.CONCLUSION HES symptoms are variable and atypical,and the level and timing of eosinophilia and organ damage are often discordant.展开更多
Idiopathic hypereosinophilic syndrome （HES） is a heterogeneous disorder characterized by hypereosinophilia and organ damage which affects men more commonly than women （9：1）. The disease can be detected incidental...Idiopathic hypereosinophilic syndrome （HES） is a heterogeneous disorder characterized by hypereosinophilia and organ damage which affects men more commonly than women （9：1）. The disease can be detected incidentally, but more often presents either insidiously or acutely with cardiac, neurological, cutaneous, pulmonary or gastro- intestinal manifestations, with cardiac involvement being a major cause of morbidity and morality in patients. 1.2 Although a major mechanism of tissue damage in HES is due to thromboembolism,3 fullblown disseminated intravascular coagulation （DIC） is apparently rare. This paper describes a man who presented acutely with life-threatening DIC complicating HES and its management, the first Chinese case reported in the literature.展开更多
BACKGROUND The incidence of pulmonary embolism(PE) in children is low, but its mortality is high. Hypereosinophilic syndrome(HES) is a group of diseases caused by an abnormal increase in eosinophilic granulocytes resu...BACKGROUND The incidence of pulmonary embolism(PE) in children is low, but its mortality is high. Hypereosinophilic syndrome(HES) is a group of diseases caused by an abnormal increase in eosinophilic granulocytes resulting in multiple-organ dysfunction. The urgent event of thromboembolism in the pulmonary region provoked by eosinophils in idiopathic HES(IHES) is relatively unusual. This article reports a case of IHES with multiple PEs and left leg venous thrombosis as the first manifestation. One month later, the patient developed Henoch-Schonlein purpura(HSP), which is very rare.CASE SUMMARY We report the case of a 12-year-old boy who was admitted to the hospital with dyspnea, left leg pain, and aggravation. He had bilateral PE and left leg venous embolism with mild eosinophilia. Low-molecular-weight heparin and urokinase were given. At the same time, the interventional department was contacted about filter implantation, followed by urokinase thrombolysis. The left leg thrombus was aspirated under ultrasound guidance. He was discharged from the hospital on rivaroxaban. One month later, he developed a rash on both legs and ankle pain consistent with HSP, with severe eosinophilia and motor and sensory disturbances. The patient was diagnosed with IHES with multiple embolisms complicated by HSP after excluding other causes of the eosinophil elevation. After glucocorticoid treatment, the symptoms were relieved, but the patient later developed purpura nephritis.CONCLUSION We report a rare and life-threatening case of IHES with multiple embolisms associated with HSP.A mild elevation of eosinophils early in the disease leads to difficulties in diagnosis and delayed treatment.展开更多
BACKGROUND Hypereosinophilia(HE)is defined as a peripheral blood eosinophil count of>1.5×109/L and may be associated with tissue damage.The clinical presentations of HE vary;however,myocardial fibrosis and thr...BACKGROUND Hypereosinophilia(HE)is defined as a peripheral blood eosinophil count of>1.5×109/L and may be associated with tissue damage.The clinical presentations of HE vary;however,myocardial fibrosis and thrombosis can threaten the lives of patients with sustained eosinophilia.Cerebral venous sinus thrombosis(CVST)in the setting of eosinophil-related diseases has seldom been reported.Here,we review the literature on HE with CVST to increase knowledge and encourage early diagnosis.CASE SUMMARY A previously healthy 41-year-old man was admitted to hospital with diarrhea and abdominal pain.He was treated with antibiotics for suspected acute colitis.Three days later,he experienced headache and vomiting.Brain computed tomography(CT)revealed thrombosis of the left jugular vein to the left transverse sinus vein.Platelet(PLT)count decreased to 60×1012/L,and absolute eosinophil count(AEC)increased to 2.41×109/L.He was treated with low-molecular-weight heparin.PLT count progressively decreased to 14×109/L,and we terminated anticoagulation and performed PLT transfusion.Six days after admission,he complained of a worsening headache.Brain CT revealed right temporal lobe and left centrum semiovale intracerebral hemorrhage,and AEC increased to 7.65×109/L.We used prednisolone for HE.The level of consciousness decreased,so emergency hematoma removal and decompressive craniectomy for right cerebral hemorrhage were performed.The patient was alert 2 d after surgery.He was treated with anticoagulation again 2 wk after surgery.Corticosteroids were gradually tapered without any symptomatic recurrence or abnormal laboratory findings.CONCLUSION HE can induce CVST,and we need to focus on eosinophil counts in patients with CVST.展开更多
Chronic eosinophilic leukemia (CEL) is a rare disorder that is characterized by hypereosinophilia with increased number of blood or marrow blasts (>5% and <20%). CEL is distinguished from hypereosinophilic syndr...Chronic eosinophilic leukemia (CEL) is a rare disorder that is characterized by hypereosinophilia with increased number of blood or marrow blasts (>5% and <20%). CEL is distinguished from hypereosinophilic syndrome (HES) by the presence of eosinophilic clonality. Chronic eosinophilic leukemia not otherwise specified (CEL-NOS) diagnosis is made when no fusion genes are detected by most modern molecular testing, particularly the most common fusion gene FIP1L-1/PDGFRA (Factor Interacting with PAP like-1/Platelet-Derived Growth Factor Receptor Alpha). This disease is very rare, and its description in the literature is not well characterized. We report a fetal case of severe CEL-NOS in a 19-year-old male who presented with a plethora of clinical features consists of constitutional symptoms, pancytopenia, intravascular thrombosis, acute stroke and endomyocardial infiltrates. The course of his disease was aggressive and resistant to conventional treatment. After a short period of improvement, an acute transformation into blast crisis (BC) had occurred. The diagnosis was confirmed by morphology and immunophenotyping of bone marrow biopsy. The patient eventually died of heart failure and sepsis. To our knowledge this is the first case report of fatal CEL-NOS transforming into severe blast crisis.展开更多
Hypereosinophilic syndrome （HES） is a rare disorder that can be seen in various organ systems, but its major tissue target is the heart.＇ The most characteristic cardiovascular abnormality in HES is endomyocardial ...Hypereosinophilic syndrome （HES） is a rare disorder that can be seen in various organ systems, but its major tissue target is the heart.＇ The most characteristic cardiovascular abnormality in HES is endomyocardial fibrosis, initially described in 1936 by Loffler, who called it ＂fibroplastic parietal endocarditis with blood eosinophilia.＂ The overall prognosis of patients with Loffler endocarditis is very poor. Current treatments include anticoagulation and anti-eosinophils therapy, and surgery only has been used in selected cases.展开更多
基金the National Science of Foundation Committee of Zhejiang Province,No.LY22H160003the Zhejiang Provincial Medical and Health Science Foundation,No.2021441200 and No.2021RC083.
文摘BACKGROUND Hypereosinophilic syndrome(HES)is classified as primary,secondary or idiopathic.Idiopathic HES(IHES)has a variable clinical presentation and may involve multiple organs causing severe damage.Hepatic sinusoidal obstruction syndrome(HSOS)is characterized by damage to the endothelial cells of the hepatic sinusoids of the hepatic venules,with occlusion of the hepatic venules,and hepatocyte necrosis.We report a case of IHES with HSOS of uncertain etiology.CASE SUMMARY A 70-year-old male patient was admitted to our hospital with pruritus and a rash on the extremities for>5 mo.He had previously undergone antiallergic treatment and herbal therapy in the local hospital,but the symptoms recurred.Relevant examinations were completed after admission.Bone marrow aspiration biopsy showed a significantly higher percentage of eosinophils(23%)with approximately normal morphology.Ultrasound-guided hepatic aspiration biopsy indicated HSOS.Contrast-enhanced computed tomography(CT)of the upper abdomen showed hepatic venule congestion with hydrothorax and ascites.The patient was initially diagnosed with IHES and hepatic venule occlusion.Prednisone,low molecular weight heparin and ursodeoxycholic acid were given for treatment,followed by discontinuation of low molecular weight heparin due to ecchymosis.Routine blood tests,biochemical tests,and imaging such as enhanced CT of the upper abdomen and pelvis were reviewed regularly.CONCLUSION Hypereosinophilia may play a facilitating role in the occurrence and development of HSOS.
文摘BACKGROUND Simultaneous cerebral and myocardial infarction with arterial involvement has not been reported in hypereosinophilic syndrome(HES).Here,we report a patient with HES that was also associated with acute ischemic stroke,myocardial infarction,and arterial involvement of the left common carotid artery,vertebral arteries,posterior cerebral artery,and coronary artery.CASE SUMMARY A 64-year-old male patient was admitted with headache and right lower extremity weakness.Laboratory tests indicated eosinophilia.Brain magnetic resonance imaging(MRI)showed bilateral and multiple acute infarcts in the border zones.Electrocardiography revealed that T wave was inverted and that the concentration of troponin I was significantly elevated above normal levels.Cardiac echocardiography showed an ejection fraction of 69%with mitral and tricuspid mild regurgitation.Computed tomography angiography detected multiple and localized instances of mild stenosis in the left common carotid artery bifurcation,bilateral vertebral arteries(V5 segment),and the posterior cerebral artery(P2 segment).These were observed together with multiple non-calcified and mixed plaques as well as luminal stenosis in the left circumflex artery,left anterior descending artery,and right coronary artery.The patient was treated with oral methylprednisolone and clopidogrel,after which the absolute eosinophil count fell rapidly to a normal level.After one month,a second brain MRI showed a partial reduction in the size and number of the lesions.CONCLUSION HES can masquerade as ischemic stroke,myocardial infarction,and arterial vascular involvement.The patient reported here recovered very quickly when his eosinophil blood count returned to normal.Early diagnosis and rapid reduction of eosinophils may lead to a good prognosis.
文摘BACKGROUND Hypereosinophilic syndrome(HES)is a condition characterized by increased eosinophil proliferation in the bone marrow,as well as tissue eosinophilia,often causing organ damage.The cause of the disease is unknown.Initial symptoms include fatigue,cough,shortness of breath,myalgia,angioedema,fever,and pneumonia.In addition to the respiratory symptoms,damage to the central nervous system can lead to severe seizures.Here,we report a case with pneumonia and complex partial seizures secondary to HES.CASE SUMMARY A 94-year-old woman was admitted to our hospital for heart failure and bloody stools.After admission,she also showed symptoms of pneumonia.Non-contrast computed tomography of the chest showed pleural effusion and infiltrative shadows.Lower gastrointestinal endoscopy showed multiple ulcers in the sigmoid colon.Blood analyses showed marked eosinophilia(eosinophils 1760/mm3,total leukocytes 6850/mm3).Initial treatment with furosemide 20 mg/d and prednisolone 25 mg/d relieved these symptoms.However,the patient subsequently experienced localised epileptic seizures characterized by bilateral eyelid twitching and eyes rolling upwards,without generalized convulsions,and respiratory arrest occurred.Electroencephalography showed spikes and waves.Non-contrast magnetic resonance imaging of the brain showed extensive periventricular hyperintensity.With administration of levetiracetam 1000 mg/d the epileptic seizures disappeared.However,the patient’s consciousness remained impaired,and her pneumonia worsened again.Two weeks later,she died of pneumonia.CONCLUSION HES symptoms are variable and atypical,and the level and timing of eosinophilia and organ damage are often discordant.
文摘Idiopathic hypereosinophilic syndrome （HES） is a heterogeneous disorder characterized by hypereosinophilia and organ damage which affects men more commonly than women （9：1）. The disease can be detected incidentally, but more often presents either insidiously or acutely with cardiac, neurological, cutaneous, pulmonary or gastro- intestinal manifestations, with cardiac involvement being a major cause of morbidity and morality in patients. 1.2 Although a major mechanism of tissue damage in HES is due to thromboembolism,3 fullblown disseminated intravascular coagulation （DIC） is apparently rare. This paper describes a man who presented acutely with life-threatening DIC complicating HES and its management, the first Chinese case reported in the literature.
文摘BACKGROUND The incidence of pulmonary embolism(PE) in children is low, but its mortality is high. Hypereosinophilic syndrome(HES) is a group of diseases caused by an abnormal increase in eosinophilic granulocytes resulting in multiple-organ dysfunction. The urgent event of thromboembolism in the pulmonary region provoked by eosinophils in idiopathic HES(IHES) is relatively unusual. This article reports a case of IHES with multiple PEs and left leg venous thrombosis as the first manifestation. One month later, the patient developed Henoch-Schonlein purpura(HSP), which is very rare.CASE SUMMARY We report the case of a 12-year-old boy who was admitted to the hospital with dyspnea, left leg pain, and aggravation. He had bilateral PE and left leg venous embolism with mild eosinophilia. Low-molecular-weight heparin and urokinase were given. At the same time, the interventional department was contacted about filter implantation, followed by urokinase thrombolysis. The left leg thrombus was aspirated under ultrasound guidance. He was discharged from the hospital on rivaroxaban. One month later, he developed a rash on both legs and ankle pain consistent with HSP, with severe eosinophilia and motor and sensory disturbances. The patient was diagnosed with IHES with multiple embolisms complicated by HSP after excluding other causes of the eosinophil elevation. After glucocorticoid treatment, the symptoms were relieved, but the patient later developed purpura nephritis.CONCLUSION We report a rare and life-threatening case of IHES with multiple embolisms associated with HSP.A mild elevation of eosinophils early in the disease leads to difficulties in diagnosis and delayed treatment.
基金Zhejiang Research Center of Stroke Diagnosis and Treatment Technology,No.JBZX-202002Zhejiang Province Medical Science and Technology Project,No.2020RC061 and No.2018KY872.
文摘BACKGROUND Hypereosinophilia(HE)is defined as a peripheral blood eosinophil count of>1.5×109/L and may be associated with tissue damage.The clinical presentations of HE vary;however,myocardial fibrosis and thrombosis can threaten the lives of patients with sustained eosinophilia.Cerebral venous sinus thrombosis(CVST)in the setting of eosinophil-related diseases has seldom been reported.Here,we review the literature on HE with CVST to increase knowledge and encourage early diagnosis.CASE SUMMARY A previously healthy 41-year-old man was admitted to hospital with diarrhea and abdominal pain.He was treated with antibiotics for suspected acute colitis.Three days later,he experienced headache and vomiting.Brain computed tomography(CT)revealed thrombosis of the left jugular vein to the left transverse sinus vein.Platelet(PLT)count decreased to 60×1012/L,and absolute eosinophil count(AEC)increased to 2.41×109/L.He was treated with low-molecular-weight heparin.PLT count progressively decreased to 14×109/L,and we terminated anticoagulation and performed PLT transfusion.Six days after admission,he complained of a worsening headache.Brain CT revealed right temporal lobe and left centrum semiovale intracerebral hemorrhage,and AEC increased to 7.65×109/L.We used prednisolone for HE.The level of consciousness decreased,so emergency hematoma removal and decompressive craniectomy for right cerebral hemorrhage were performed.The patient was alert 2 d after surgery.He was treated with anticoagulation again 2 wk after surgery.Corticosteroids were gradually tapered without any symptomatic recurrence or abnormal laboratory findings.CONCLUSION HE can induce CVST,and we need to focus on eosinophil counts in patients with CVST.
文摘Chronic eosinophilic leukemia (CEL) is a rare disorder that is characterized by hypereosinophilia with increased number of blood or marrow blasts (>5% and <20%). CEL is distinguished from hypereosinophilic syndrome (HES) by the presence of eosinophilic clonality. Chronic eosinophilic leukemia not otherwise specified (CEL-NOS) diagnosis is made when no fusion genes are detected by most modern molecular testing, particularly the most common fusion gene FIP1L-1/PDGFRA (Factor Interacting with PAP like-1/Platelet-Derived Growth Factor Receptor Alpha). This disease is very rare, and its description in the literature is not well characterized. We report a fetal case of severe CEL-NOS in a 19-year-old male who presented with a plethora of clinical features consists of constitutional symptoms, pancytopenia, intravascular thrombosis, acute stroke and endomyocardial infiltrates. The course of his disease was aggressive and resistant to conventional treatment. After a short period of improvement, an acute transformation into blast crisis (BC) had occurred. The diagnosis was confirmed by morphology and immunophenotyping of bone marrow biopsy. The patient eventually died of heart failure and sepsis. To our knowledge this is the first case report of fatal CEL-NOS transforming into severe blast crisis.
文摘Hypereosinophilic syndrome （HES） is a rare disorder that can be seen in various organ systems, but its major tissue target is the heart.＇ The most characteristic cardiovascular abnormality in HES is endomyocardial fibrosis, initially described in 1936 by Loffler, who called it ＂fibroplastic parietal endocarditis with blood eosinophilia.＂ The overall prognosis of patients with Loffler endocarditis is very poor. Current treatments include anticoagulation and anti-eosinophils therapy, and surgery only has been used in selected cases.
