Background:Hypereosinophilia(HE)is a rare disease characterized by an increase in eosinophils.Suplatast tosilate is a selective Th2 cytokine inhibitor.This case report presents the course and prognosis of a patient wi...Background:Hypereosinophilia(HE)is a rare disease characterized by an increase in eosinophils.Suplatast tosilate is a selective Th2 cytokine inhibitor.This case report presents the course and prognosis of a patient with hypereosinophilia treated with suplatast tosilate monotherapy.Case presentation:A 41-year-old female patient who complained of“Elevated blood eosinophils were found during physical examination for more than 2 months”visited the Allergy Department.The systematic screening results of parasites,autoantibodies spectrum,tumor markers,peripheral blood morphology analysis,and leukemia fusion gene(FIP1L1-PDGFRα)were all negative.Gastrointestinal endoscopy only indicated mild gastritis.The dust mites and multiple fungal mixtures were weakly positive in intradermal tests.Finally diagnosed HE.After treatment with suplatast tosilate 2 months of 100mg three times daily,the blood eosinophils decreased to normal.Then,maintenance treatment with 50mg three times a day for 3 months.And without recurrence during nearly five years of follow-up.Conclusion:The report indicates that suplatast tosilate can reduce peripheral blood eosinophils levels in patients with HE,and may be a reliable option for individualized treatment of HE.However,there is still no consensus on whether suplatast tosilate can be used as a first-line treatment or as an adjuvant therapy to reduce the corticosteroids dosage for patients with eosinophilia,and further research is needed.展开更多
BACKGROUND Hypereosinophilia(HE)is a heterogeneous disease of unknown etiology in which tissue and organ injury is inflicted by excess numbers of circulating or infiltrating eosinophils.Herein,we describe a patient wi...BACKGROUND Hypereosinophilia(HE)is a heterogeneous disease of unknown etiology in which tissue and organ injury is inflicted by excess numbers of circulating or infiltrating eosinophils.Herein,we describe a patient with rare organ damage due to HE and review the pertinent literature.CASE SUMMARY A 43 year-old Chinese man with a 13-year history of eosinophilia and shortness of breath for 7 d presented to our hospital.During the course of his illness,the patient variably presented with gastrointestinal symptoms,eczema,vitiligo,mastitis,joint symptoms,nephrotic syndrome,and interstitial pneumonia.The chronic mastitis proved burdensome,necessitating bilateral mastectomy.HE was diagnosed by repeat bone marrow biopsy,and a kidney biopsy showed focal segmental glomerulosclerosis.Intermittent steroidal therapy is typically initiated to relieve such symptoms,although relapse and organ involvement often ensue once treatment is withdrawn.We administered methylprednisolone sodium succinate(40 mg/d)intravenously for 3 d,followed by oral tablets at the same dose.Subsequent computed tomography(CT)of the chest CT showed relative improvement of the interstitial pneumonia.The patient is currently on a continuous regimen of oral steroid,and his condition is stable.CONCLUSION HE is heterogeneous condition.This is the first reported case of bilateral mastectomy in a male patient with longstanding HE.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
Objective To investigate the Churg-Strauss syndrome （CSS） associated lung involvement, concentrating on clinical characteristics, pathological findings of tung involvements, response to treatment, and prognosis. Me...Objective To investigate the Churg-Strauss syndrome （CSS） associated lung involvement, concentrating on clinical characteristics, pathological findings of tung involvements, response to treatment, and prognosis. Methods We retrospectively analyzed the characters of the clinical manifestations, thin-section CT and pathological findings of CSS. The study involved 16 patients. Clinical data were obtained by chart review All patients underwent transbronchial lung biopsy （TBLB）. Six of them underwent surgical lung biopsy as well. Results The patients included 7 men and 9 women, aged from 14 to 61 years （median, 47.5 years）. Extrathoracic organs involved included nervous system （7/16） and skin （5/16）. Respiratory symptoms included cough （12/16）, exertional dyspnea （11/16）, hemoptysis （4/16）, and chest pain （3/16）. CT findings included bilateral ground-glass opacities （12 / 16）, bilateral patchy opacities （12 / 16）, and centrilobular nodules （6/16）. The pathological findings of TBLB demonstrated increased eosinophils （3/16）, vasculitis （3/16）, and interstitial pneumonia （16/16）. The pathological fmdings of surgical lung biopsy of 6 cases showed necrotizing vasculitis in 4 cases, capillaries in 5, eosinophilic pneumonia in 3, granulomas in 2, and airway abnormalities in 3. All patients improved in symptoms after therapy during the study period （range, 3 to 51 months; median, 15 months）. Conclusions Asthma may be present in CSS patient when there is bronchial involvement. Grotmd-glass opacities and consolidation seen on high-resolution CT reflect the presence of eosinophilic pneumonia, vasculitis, and pulmonary alveolar hemorrhage. TBLB has significant limitations for the diagnosis of CSS. Early diagnosis and therapy can result in satisfactory prognosis.展开更多
In this paper we present a five-generation kindred with familial eosinophilia, associated with valvular heart disease in one of seven members of the second generation, four of sixteen members of the third generation, ...In this paper we present a five-generation kindred with familial eosinophilia, associated with valvular heart disease in one of seven members of the second generation, four of sixteen members of the third generation, four of thirty-one members of the fourth generation, and two of twenty members of the fifth generation;the clinical features of the two members of the first generation are unavailable. Of a total of 48 members, 26 had eosinophilia, with counts ranging from 1761 to 6045 cells/mm<sup>3</sup>, with apparent autosomal dominant propagation;this confirms the experience of the authors of previous studies on this condition. Genetic analysis on eight members, which we reported in an earlier paper [1], revealed a pericentric inversion of chromosome 10 in two members. The entity of Familial Eosinophilia has been generally considered benign. However, the clinical presentation of this kindred was unusual in that valvular and endocardial damage, was frequent findings, without apparent involvement of the other organs and tissues. Mitral valve damage leading to both stenosis and regurgitation and requiring mitral valve replacement was noted in the index patient. This unique presentation may suggest that in patients with mitral valve disease, if blood eosinophilia is noted, it could point to a non-rheumatic etiology, thus a possible opportunity for treatment to prevent further damage to the heart. This recommendation may be even more timely, as many effective treatments are now available to treat even high-grade hypereosinophilia.展开更多
背景嗜酸性粒细胞增多综合征(hypereosinophilia syndrome,HES)是一组以血液和(或)组织中嗜酸性粒细胞(eosinophil,EOS)异常增多为特征的疾病,临床表现多样,易漏诊或误诊为其他伴嗜酸性粒细胞增多的皮炎瘙痒性疾病,其中特别需与特应性皮...背景嗜酸性粒细胞增多综合征(hypereosinophilia syndrome,HES)是一组以血液和(或)组织中嗜酸性粒细胞(eosinophil,EOS)异常增多为特征的疾病,临床表现多样,易漏诊或误诊为其他伴嗜酸性粒细胞增多的皮炎瘙痒性疾病,其中特别需与特应性皮炎(atopic dermatitis,AD)进行鉴别诊断。目的评估外周血EOS计数在嗜酸性粒细胞增多综合征与特应性皮炎鉴别中的作用。方法2015年1月—2022年12月解放军总医院第一医学中心皮肤科收治的诊断明确的HES和AD患者为样本,通过受试者工作特征曲线法计算曲线下面积并评估外周血EOS的鉴别诊断效能。2023年6—12月收治的诊断明确的HES患者和外周血EOS计数>0.5×10^(9)/L的AD患者为后续研究对象,比较二者非激素系统治疗2周内外周血EOS计数变化趋势。结果HES组58例,男49例,女9例,中位年龄46(IQR:23~67)岁;AD组133例,男74例,女59例,中位年龄55(IQR:38~69)岁;HES组患者年龄更大、男性比例更高(P<0.05)。HES组中位白细胞计数[M(IQR):10.78(8.97~13.75)×10^(9)/L vs 6.61(5.44~8.55)×10^(9)/L]和中位外周血嗜酸性粒细胞计数[M(IQR):3.29(1.77~5.15)×10^(9)/L vs 0.60(0.35~1.05)×10^(9)/L]均高于AD组,差异有统计学意义(P<0.05)。HES鉴别AD诊断预测模型的ROC曲线结果提示:EOS鉴别诊断最佳阈值为1.52×10^(9)/L,敏感度为83.2%,特异度为91.8%。后续研究中10例HES患者,治疗前EOS均值(4.51±1.22)×10^(9)/L,给予2周非激素系统治疗,治疗后EOS均值(5.56±1.31)×10^(9)/L,无显著下降趋势。10例AD患者,经非激素系统治疗后第4~8天外周血EOS可降至正常水平(<0.5×10^(9)/L),下降程度平均值为(72.10%±17.12%)。结论HES和AD患者外周血EOS计数分布范围不等,且大部分AD患者给予非激素系统治疗后短期内外周血EOS计数可显著下降,可为HES与AD初步鉴别诊断提供参考,迅速启动干预治疗,对HES在不可逆的器官损伤发生之前减少EOS浸润,进而降低器官损伤的风险。展开更多
基金supported by the National High-Level Hospital Clinical Research Funding(2022-PUMCH-D-002).
文摘Background:Hypereosinophilia(HE)is a rare disease characterized by an increase in eosinophils.Suplatast tosilate is a selective Th2 cytokine inhibitor.This case report presents the course and prognosis of a patient with hypereosinophilia treated with suplatast tosilate monotherapy.Case presentation:A 41-year-old female patient who complained of“Elevated blood eosinophils were found during physical examination for more than 2 months”visited the Allergy Department.The systematic screening results of parasites,autoantibodies spectrum,tumor markers,peripheral blood morphology analysis,and leukemia fusion gene(FIP1L1-PDGFRα)were all negative.Gastrointestinal endoscopy only indicated mild gastritis.The dust mites and multiple fungal mixtures were weakly positive in intradermal tests.Finally diagnosed HE.After treatment with suplatast tosilate 2 months of 100mg three times daily,the blood eosinophils decreased to normal.Then,maintenance treatment with 50mg three times a day for 3 months.And without recurrence during nearly five years of follow-up.Conclusion:The report indicates that suplatast tosilate can reduce peripheral blood eosinophils levels in patients with HE,and may be a reliable option for individualized treatment of HE.However,there is still no consensus on whether suplatast tosilate can be used as a first-line treatment or as an adjuvant therapy to reduce the corticosteroids dosage for patients with eosinophilia,and further research is needed.
文摘BACKGROUND Hypereosinophilia(HE)is a heterogeneous disease of unknown etiology in which tissue and organ injury is inflicted by excess numbers of circulating or infiltrating eosinophils.Herein,we describe a patient with rare organ damage due to HE and review the pertinent literature.CASE SUMMARY A 43 year-old Chinese man with a 13-year history of eosinophilia and shortness of breath for 7 d presented to our hospital.During the course of his illness,the patient variably presented with gastrointestinal symptoms,eczema,vitiligo,mastitis,joint symptoms,nephrotic syndrome,and interstitial pneumonia.The chronic mastitis proved burdensome,necessitating bilateral mastectomy.HE was diagnosed by repeat bone marrow biopsy,and a kidney biopsy showed focal segmental glomerulosclerosis.Intermittent steroidal therapy is typically initiated to relieve such symptoms,although relapse and organ involvement often ensue once treatment is withdrawn.We administered methylprednisolone sodium succinate(40 mg/d)intravenously for 3 d,followed by oral tablets at the same dose.Subsequent computed tomography(CT)of the chest CT showed relative improvement of the interstitial pneumonia.The patient is currently on a continuous regimen of oral steroid,and his condition is stable.CONCLUSION HE is heterogeneous condition.This is the first reported case of bilateral mastectomy in a male patient with longstanding HE.
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Objective To investigate the Churg-Strauss syndrome （CSS） associated lung involvement, concentrating on clinical characteristics, pathological findings of tung involvements, response to treatment, and prognosis. Methods We retrospectively analyzed the characters of the clinical manifestations, thin-section CT and pathological findings of CSS. The study involved 16 patients. Clinical data were obtained by chart review All patients underwent transbronchial lung biopsy （TBLB）. Six of them underwent surgical lung biopsy as well. Results The patients included 7 men and 9 women, aged from 14 to 61 years （median, 47.5 years）. Extrathoracic organs involved included nervous system （7/16） and skin （5/16）. Respiratory symptoms included cough （12/16）, exertional dyspnea （11/16）, hemoptysis （4/16）, and chest pain （3/16）. CT findings included bilateral ground-glass opacities （12 / 16）, bilateral patchy opacities （12 / 16）, and centrilobular nodules （6/16）. The pathological findings of TBLB demonstrated increased eosinophils （3/16）, vasculitis （3/16）, and interstitial pneumonia （16/16）. The pathological fmdings of surgical lung biopsy of 6 cases showed necrotizing vasculitis in 4 cases, capillaries in 5, eosinophilic pneumonia in 3, granulomas in 2, and airway abnormalities in 3. All patients improved in symptoms after therapy during the study period （range, 3 to 51 months; median, 15 months）. Conclusions Asthma may be present in CSS patient when there is bronchial involvement. Grotmd-glass opacities and consolidation seen on high-resolution CT reflect the presence of eosinophilic pneumonia, vasculitis, and pulmonary alveolar hemorrhage. TBLB has significant limitations for the diagnosis of CSS. Early diagnosis and therapy can result in satisfactory prognosis.
文摘In this paper we present a five-generation kindred with familial eosinophilia, associated with valvular heart disease in one of seven members of the second generation, four of sixteen members of the third generation, four of thirty-one members of the fourth generation, and two of twenty members of the fifth generation;the clinical features of the two members of the first generation are unavailable. Of a total of 48 members, 26 had eosinophilia, with counts ranging from 1761 to 6045 cells/mm<sup>3</sup>, with apparent autosomal dominant propagation;this confirms the experience of the authors of previous studies on this condition. Genetic analysis on eight members, which we reported in an earlier paper [1], revealed a pericentric inversion of chromosome 10 in two members. The entity of Familial Eosinophilia has been generally considered benign. However, the clinical presentation of this kindred was unusual in that valvular and endocardial damage, was frequent findings, without apparent involvement of the other organs and tissues. Mitral valve damage leading to both stenosis and regurgitation and requiring mitral valve replacement was noted in the index patient. This unique presentation may suggest that in patients with mitral valve disease, if blood eosinophilia is noted, it could point to a non-rheumatic etiology, thus a possible opportunity for treatment to prevent further damage to the heart. This recommendation may be even more timely, as many effective treatments are now available to treat even high-grade hypereosinophilia.
文摘背景嗜酸性粒细胞增多综合征(hypereosinophilia syndrome,HES)是一组以血液和(或)组织中嗜酸性粒细胞(eosinophil,EOS)异常增多为特征的疾病,临床表现多样,易漏诊或误诊为其他伴嗜酸性粒细胞增多的皮炎瘙痒性疾病,其中特别需与特应性皮炎(atopic dermatitis,AD)进行鉴别诊断。目的评估外周血EOS计数在嗜酸性粒细胞增多综合征与特应性皮炎鉴别中的作用。方法2015年1月—2022年12月解放军总医院第一医学中心皮肤科收治的诊断明确的HES和AD患者为样本,通过受试者工作特征曲线法计算曲线下面积并评估外周血EOS的鉴别诊断效能。2023年6—12月收治的诊断明确的HES患者和外周血EOS计数>0.5×10^(9)/L的AD患者为后续研究对象,比较二者非激素系统治疗2周内外周血EOS计数变化趋势。结果HES组58例,男49例,女9例,中位年龄46(IQR:23~67)岁;AD组133例,男74例,女59例,中位年龄55(IQR:38~69)岁;HES组患者年龄更大、男性比例更高(P<0.05)。HES组中位白细胞计数[M(IQR):10.78(8.97~13.75)×10^(9)/L vs 6.61(5.44~8.55)×10^(9)/L]和中位外周血嗜酸性粒细胞计数[M(IQR):3.29(1.77~5.15)×10^(9)/L vs 0.60(0.35~1.05)×10^(9)/L]均高于AD组,差异有统计学意义(P<0.05)。HES鉴别AD诊断预测模型的ROC曲线结果提示:EOS鉴别诊断最佳阈值为1.52×10^(9)/L,敏感度为83.2%,特异度为91.8%。后续研究中10例HES患者,治疗前EOS均值(4.51±1.22)×10^(9)/L,给予2周非激素系统治疗,治疗后EOS均值(5.56±1.31)×10^(9)/L,无显著下降趋势。10例AD患者,经非激素系统治疗后第4~8天外周血EOS可降至正常水平(<0.5×10^(9)/L),下降程度平均值为(72.10%±17.12%)。结论HES和AD患者外周血EOS计数分布范围不等,且大部分AD患者给予非激素系统治疗后短期内外周血EOS计数可显著下降,可为HES与AD初步鉴别诊断提供参考,迅速启动干预治疗,对HES在不可逆的器官损伤发生之前减少EOS浸润,进而降低器官损伤的风险。
