Idiopathic hypereosinophilic syndrome with hepatic sinusoidal obstruction syndrome:A case report and literature review

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.

Hypereosinophilic syndrome presenting as acute ischemic stroke,myocardial infarction,and arterial involvement:A case report

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.

Pneumonia and seizures due to hypereosinophilic syndrome—organ damage and eosinophilia without synchronisation:A case report

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.

A case of hypereosinophilic syndrome presenting with intractable gastric ulcers

We report a rare case of hypereosinophilic syndrome （HES） presenting with intractable gastric ulcers. A 71-year-old man was admitted with epigastric pain. Initial endoscopic findings revealed multiple, active gastric ulcers in the gastric antrum. He underwent Helicobacterpylori （Hpylon） eradication therapy followed by proton pump inhibitor （PPI） therapy. However, follow- up endoscopy at 4, 6, 10 and 14 mo revealed persistent multiple gastric ulcers without significant improvement. The proportion of his eosinophil count increased to 43% （total count： 7903/mm3）. Abdominal-pelvic and chest computed tomography scans showed multiple small nodules in the liver and both lungs. The endoscopic biopsy specimen taken from the gastric antrum revealed prominent eosinophilic infiltration, and the liver biopsy specimen also showed eosinophilic infiltration in the portal tract and sinusoid. A bone marrow biopsy disclosed eosinophilic hyperplasia as well as increased cellularity of 70%. The patient was finally diagnosed with HES involving the stomach, liver, lung, and bone marrow. When gastric ulcers do not improve despite H pylon eradication and prolonged PPI therapy, infiltrative gastric disorders such as HES should be considered.

Spontaneous liver rupture in hypereosinophilic syndrome:A rare but fatal complication

We report a rare case of spontaneous liver rupture in a patient with hypereosinophilic syndrome(HES),of which the diagnosis was delayed,resulting in a fatal outcome.The diagnostic criteria and treatment of HES with hepatic involvement were reviewed.The possible cause of spontaneous liver rupture in HES and its management were also discussed.To our knowledge,this is the fi rst case report of spontaneous liver rupture in HES.We emphasized the need of a high index of suspicion in diagnosing HES,so that early treatment could be initiated.

Idiopathic hypereosinophilic syndrome presenting with severe vasculitis successfully treated with imatinib

Idiopathic hypereosinophilic syndrome(HES) is a rare disorder characterized by peripheral eosinophilia exceeding 1500/mm3, a chronic course, absence of secondary causes, and signs and symptoms of eosinophil-mediated tissue injury. One of the best-characterized forms of HES is the one associated with FIP1L1-PDGFRA gene rearrangement, which was recently demonstrated as responsive to treatment with the small molecule kinase inhibitor drug, imatinib mesylate. Here, we describe the case of a 51-year-old male, whose symptoms satisfied the clinical criteria for HES with cutaneous and cardiac involvement and who also presented with vasculitic brain lesions and retroperitoneal bleeding. Molecular testing, including fluorescence in situ hybridization, of bone marrow and peripheral blood showed no evidence of PDGFR rearrangements. The patient was initially treated with high-dose steroid therapy and then with hydroxyurea, but proved unresponsive to both. Upon subsequent initiation of imatinib mesilate, the patient showed a dramatic improvement in eosinophil count and progressed rapidly through clinical recovery. Long-term follow-up confirmed the efficacy of treatment with low-dose imatinib and with no need of supplemental steroid treatment, notwithstanding the absence of PDGFR rearrangement.

Idiopathic Hypereosinophilic Syndrome Presenting as IgA Nephropathy with Nephrotic Range Proteinuria

Idiopathic hypereosinophilic syndrome (IHES) is a disorder characterized by increased eosinophil count (eosinophilia) along with organ dysfunction secondary to organ infiltration of eosinophils and release of inflammatory markers [1-4], with no obvious cause for eosinophilia. The onset of symptoms is insidious in most of the cases and eosinophilia is detected incidentally. However, in others, the initial manifestations are severe and life-threatening due to the rapid evolution of cardiac or neurologic complications [5]. Renal involvement is rarely reported [6] in IHES. Herein we reported a case of IHES with predominant renal involvement as nephrotic syndrome with focal necrotizing IgA nephropathy.

Type I Neurofibromatosis Associated with Hypereosinophilic Syndrome: A Rare Case Report

Introduction Hypereosinophilic syndrome (HES) encompasses a group of disorders characterized by marked peripheral blood eosinophilia,tissue eosinophilia,or both,resulting in organ damage attributable to the eosinophilia.Type Ⅰ neurofibromatosis (NF-I) is an autosomal dominant genodermatosis with an increased risk of developing malignancies.1 HES was also considered as paraneoplastic presentation and it is proved to arise in combination with hematological malignancies such as chronic myeloid leukemia (CML),acute lymphocytic leukemia (ALL),and non-Hodgkin lymphoma (NHL).2 Here,we have reported a rare case of HES associated with NF-I that has not been reported so far,which may improve the knowledge of association between NF-1 and hematological malignancies.

An unusual case of hypereosinophilic syndrome and disseminated intravascular coagulation

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.

Development of Henoch-Schoenlein purpura in a child with idiopathic hypereosinophilia syndrome with multiple thrombotic onset: A case report

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.

Hypereosinophilia with cerebral venous sinus thrombosis and intracerebral hemorrhage:A case report and review of the literature

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.

Assessment of Molecular Signalling Mechanisms for Eosinophilia in Rottweilers

Rottweilers are predisposed to eosinophilic diseases, including hypereosinophilic syndrome. The immunopathogenesis of idiopathic eosinophilia is poorly characterised in dogs and man. Studies in people have suggested cytokines, particularly interleukin (IL)-5, play a role in instigating and perpetuating eosinophilia. This study sought to establish whether differences in gene expression, and concentration of selected, cytokines and chemokines were associated with eosinophilia in Rottweilers. Quantitative real-time reverse transcriptase polymerase chain reaction (qRT-PCR) assays were used to quantify messenger ribonucleic acid (mRNA) encoding cytokines IL-4, -5, -10, -12p19, -12p35, -12p40, -18, interferon gamma (IFN-γ) and chemokines eotaxin-2 and -3 from peripheral blood mononuclear cell (PMBC) samples obtained from healthy dogs (breeds other than Rottweiler) with normal eosinophil blood counts (n = 5) and Rottweilers with normal (n = 6), mildly increased (n = 7) and high (n = 3) eosinophil blood counts. Quantification of plasma IFN-γ and IL-5 was performed using commercially available canine-specific enzyme-linked immunosorbent assays ELISAs. Cytokine mRNA was measurable in all samples, although eotaxin-2 and -3 were not detected. No significant differences in gene expression of any cytokine were found between groups (based on eosinophil count or breed). No significant difference in plasma IL-5 or IFN-γ concentration was present between groups. In conclusion, there were no significant differences in cytokine mRNA profiles or plasma IL-5 and IFN-γ levels between Rottweilers with increased eosinophil counts and Rottweiler and non-Rottweiler dogs with normal eosinophil counts.

A Fatal Case of Chronic Eosinophilic Leukemia Not Otherwise Specified (CEL-NOS) in a 19-Year-Old Male with Acute Transformation into Blast Crisis

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.

儿童高嗜酸粒细胞增多症并发Loffler心内膜炎致死1例

目的探讨儿童高嗜酸粒细胞增多症(hypereosinophilia,HE)并发Loffler心内膜炎的临床特征,旨在分析HE并发Loffler心内膜炎的病因、临床分期及预后。方法回顾性分析河北医科大学第二医院诊治的1例儿童高嗜酸粒细胞增多症并发Loffler心内膜炎的病例,分析其临床特点及发生原因及临床分期。结果本研究中该病例为少年男性,因间断腹痛腹泻40余天,呼吸困难10 d,进食极差3 d就诊,血常规提示为HE,超声提示Loffler心内膜炎,患儿存在严重心力衰竭,多脏器功能受累,经积极治疗后仍出现多脏器功能衰竭,最终因患者出现多脏器衰竭、心室颤动死亡。结论Loffler心内膜炎是HE的严重并发症,发病率低,临床罕见,早期干预可降低病死率。

Hypereosinophilia with a giant thrombus in the right ventricle： Loffler endocarditis in an ll-year-old girl

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.