Hypereosinophilic syndrome: a rare cause of ST-elevation myocardial infarction and thrombus formation on the aortic valve.

We present a case of a man in his 30s presenting with ST-segment elevation myocardial infarction and eosinophilia. The patient underwent thrombus aspiration and initially echocardiographic evaluation was normal. The patient was discharged after 2 days, but was hospitalised again after 6 days. Echocardiographic evaluation now revealed a thrombus formation on the aortic valve. Laboratory data revealed increasing eosinophilia, and treatment with high-dosage corticosteroids and hydroxyurea was initiated as eosinophilic disease with organ manifestations could not be precluded. Eosinophils normalised and the patient was discharged again. The combination of hypereosinophilia and absence of infection, rheumatological disorders and malignancy, led to reactive or idiopathic hypereosinophilic syndrome being the most plausible diagnoses. The patient was closely monitored in the cardiology and haematology outpatient clinics. Echocardiographic evaluation, performed 6 weeks after the patient was discharged, showed significant regression in the size of the thrombus mass.

An 85-Year-Old Man with Fever, Dyspnea, and Dry Cough Diagnosed with Idiopathic Hypereosinophilic Syndrome, Successfully Treated with High-Dose Corticosteroids.

BACKGROUND Idiopathic hypereosinophilic syndrome (I-HES) is a rare disease diagnosed as absolute eosinophil count >1500 cells/µl and end-organ involvement attributable to tissue eosinophilia with no secondary cause of underlying eosinophilia. The mean age of presentation for I-HES is 44 years. The skin, lungs, and gastrointestinal (GI) system are most common sites of presenting manifestations, including fatigue, cough, dyspnea, myalgias, angioedema, rash, fever, nausea, and diarrhea. Although cardiac and neurologic symptoms are less common at presentation, they can be life-threatening. CASE REPORT We report the case of an 85-year-old man who presented with fever, malaise, and loss of appetite for 3 weeks, followed by dyspnea and dry cough for 2 weeks. His absolute eosinophil count was 9000 cells/µl, which was not responding to empirical antibiotic therapy, with worsening of symptoms, suggesting a non-infective origin. He was then extensively evaluated to establish underlying an etiology for specific treatment, which was negative for common causes like atypical infections, malignancy, and autoimmune disorders. He was then started on corticosteroid therapy to overcome an exaggerated immune response and reduce inflammation-related injury, to which he responded well. On follow-up, hypereosinophilia was fully cured, with reversal of end-organ involvement including myocarditis and pneumonitis. CONCLUSIONS This report shows that idiopathic HES can present with various clinical features and that accurate diagnosis, excluding known causes of eosinophilia, and early management are essential to prevent long-term organ damage. Our patient responded to prompt treatment with high-dose corticosteroids.

[Chronic neutrophilic leukemia/chronic eosinophilic leukemia].

Chronic neutrophilic leukemia (CNL) is a clonal disorder that is characterized by increasing mature neutrophils. Colony stimulating factor 3 receptor (CSF3R) T618I mutation was frequently identified in patients with CNL and is defined as a molecular marker of the disease. Ruxolitinib, a JAK2 inhibitor, provided a promising therapeutic effect in a phase II study. In particular, ruxolitinib was more efficient for patients with CSF3R mutation. Allogeneic stem cell transplantation (Allo-SCT) may be a curative treatment for CNL. On the other hand, further studies are needed to define the optimal method of transplantation, source of donor, conditioning therapy, and timing of transplantation. Chronic eosinophilic leukemia (CEL) is a clonal disorder that is characterized by increasing eosinophils. In the World Health Organization Classification 5th edition, diagnostic criteria for CEL are renewed. Because the new criteria will be more specific for CEL than criteria in the older edition, "not otherwise specified (NOS) " is removed from the name of the disease. Anti-CD52 antibody, alemtuzumab, or anti-IL-5 antibody, mepolizumab, are promising drugs to control symptoms that are associated with hypereosinophilic syndrome. Allo-SCT is anticipated as a curative treatment for CEL, but the evidence of Allo-SCT for CEL is still limited. Further study is required to define the treatment strategy.

Hypereosinophilic syndrome with massive liver infarction: A case report.

RATIONALE: Liver infarction caused only by hepatic artery occlusion is rare. Elevated levels of eosinophils in the blood and tissue can have devastating consequences. PATIENT CONCERNS: Male, 21 years old, presented with persistent abdominal distension and discomfort for more than ten days without an apparent cause. Laboratory findings showed an eosinophil percentage of 32.5% (normal range 0.5%-5%). Computed tomographic angiography of the hepatic artery and its branches did not show any enhancement, only the common hepatic artery was visible. DIAGNOSIS: The patient in this case had a peripheral blood eosinophil count of ≥1.5 × 109/L in multiple examinations over 6 months, and eosinophilic leukemia and secondary causes such as parasitic infections, allergic diseases, or tumors were ruled out, confirming the diagnosis of hypereosinophilic syndrome (HES). INTERVENTIONS: The patients were treated with interventional therapy, glucocorticoid pulse therapy and anti-infection therapy. OUTCOMES: After interventional therapy, glucocorticoid pulse therapy, and anti-infection treatment, the patient was reexamined 2 months later. The CT scan showed that the range of the original infarction in the liver had shrunk compared to before, and the remaining liver had enlarged with good compensation; Laboratory tests improved compared with baseline: eosinophil percentage of 0.1%. LESSONS: This article discusses a rare case of hepatic artery occlusion and liver infarction in a young male patient with HES. The cause of hepatic artery embolism and hepatic infarction may be related to the abnormal increase in eosinophils, which can lead to hypercoagulation and thrombus formation. The article emphasizes the importance of timely diagnosis and treatment of HES to prevent life-threatening thrombotic events and describes the successful management of the patient condition through anticoagulation, anti-infection, liver protection, and glucocorticoid therapy.

Idiopathic hypereosinophilic syndrome presenting as capsular warning syndrome: A case report and literature review.

RATIONALE: Few reports of idiopathic hypereosinophilic syndrome exist presenting as ischemic cerebrovascular disease, and the majority are watershed infarction. We report the first case of idiopathic hypereosinophilic syndrome that has clinical features of capsular warning syndrome lasting 6 weeks. PATIENT CONCERNS: A 26-year-old man complained of recurrent right limb weakness, accompanying slurred speech, and right facial paresthesia. DIAGNOSES: The patient was diagnosed with idiopathic hypereosinophilic syndrome (IHES). INTERVENTIONS: Adequate glucocorticoid and anticoagulant treatments were given. OUTCOMES: The patient's motor ability improved, and he was discharged 2 weeks later. Muscle strength in the right-side extremities had fully recovered at a 3-month follow-up after discharge. LESSONS: This case suggests that idiopathic hypereosinophilic syndrome should be considered as a cause of capsular warning syndrome, and the dose of glucocorticoid and the efficacy evaluation index needs to be reevaluated for the treatment of ischemic cerebrovascular disease associated with idiopathic hypereosinophilic syndrome.

Buddi-Chiari syndrome associated with hypereosinophilic syndrome: A case report.

RATIONALE: Budd-Chiari Syndrome (BCS) is a relatively rare clinical disorder with a wide range of symptoms, caused by the obstruction of the hepatic venous outflow. The etiology and pathogenesis of BCS vary in different countries and regions. In Western countries, hepatic venous obstruction is the most common type, and its main cause is closely related to the hypercoagulable state of the body. Inferior vena cava obstruction is common in Asia, and its etiology progresses slowly due to the lack of epidemiological data. [3] Here, we report a rare case of BCS associated with the hypereosinophilic syndrome and discuss the possible causal relationship between the two. PATIENT CONCERNS: The patient was a 33-year-old female with intermittent epistaxis, gum bleeding, and excessive menstrual flow for the past 6 months. The routine blood tests showed elevated levels of eosinophils, and the liver function test showed mildly elevated levels of γ-glutamyl transpeptidase and alkaline phosphatase, and abdominal ultrasound showed hepatosplenomegaly and suspicion of intrahepatic arteriovenous or arteriovenous-portal fistula. DIAGNOSES: Finally, through the improvement of bone marrow aspiration, digital subtraction angiography and gene detection, the diagnosis of BCS combined with hypereosinophilic syndrome was confirmed, and JAK2V617F mutation was highly associated with it. INTERVENTIONS: The patient received endovascular stent implantation and regular oral rivaroxaban anticoagulation therapy after operation. OUTCOMES: Seven months later, enhanced computed tomography (CT) of the hepatobiliary showed that the hepatic bruise-like changes were significantly reduced compared with before, and the right hepatic vein and the right perihepatic vein stent were left in place with a good filling of contrast in the stent. LESSONS: The patient, in this case, was finally diagnosed with BCS combined with hypereosinophilic syndrome, and to our knowledge, such case reports are rare. Our case report suggest an association between BCS and hypereosinophilic syndrome, but relevant studies are minimal, we hope to conduct larger and higher quality studies on these patients in the future, to provide new directions and basis for the etiology and pathogenesis of these diseases, as well as provide new targets and ideas for clinical treatment.

The portrait of a stranger: the hypereosinophilic syndrome with cardiac involvement.

INTRODUCTION: Hypereosinophilic syndrome is a rare clinical condition, and cardiac involvement confers a poor prognosis. Hypereosinophilic myocarditis is a medical emergency and targeted treatment should be started promptly even before a definitive diagnosis could be made. CASE PRESENTATION: A 27-year-old female patient is hospitalized for exertional dyspnea, chest pain, and fatigue for the past 2 weeks. She also describes left leg paresthesias. Clinical examination was in normal limits. ECG showed sinus tachycardia, QS pattern in V1-V4, and diffuse flattened T waves. Laboratory tests revealed increased inflammatory markers, hypereosinophilia, elevated cardiac enzymes, high NT-proBNP. Echocardiography revealed LV dysfunction (EF 31%), while cardiac MRI showed diffuse delayed enhancement with predominant subendocardial disposition. The electromyogram was suggestive of left tibial nerve neuropathy. We interpreted the case as eosinophilic myocarditis with an urgent requirement of therapy and initiated high-dose glucocorticoid therapy and the GDMT 4-pillar heart failure treatment. We excluded common infectious, myeloproliferative syndromes, and frequent associated autoimmune diseases. With prednisone, the eosinophil count rapidly normalized and we gradually tapered the dose by 5 mg per week, however continuing with heart failure therapy. At monthly follow-up visits, there was a significant clinical improvement, with normalization of the eosinophilic count, and a near-normalization of myocardial function. The only symptom that persisted was paresthesias linked to left tibial neuropathy. CONCLUSION: The surprisingly rapid and favorable course of the disease offers a high index of suspicion for a toxic or a reactive transitory etiology, however still unidentified. In our case, the cause of eosinophilia remained unknown, although we managed to narrow down the possible etiologies. A surprisingly good clinical response was obtained with non-specific treatment targeting mainly hyperosinophilic myocarditis.

Eosinophilic myocarditis: comprehensive update on pathophysiology, diagnosis, prognosis and management.

Hypereosinophilic syndromes are a group of disorders secondary to the accumulation of eosinophils leading to the injury of one or more organs. Among them, eosinophilic myocarditis (EM) is a rare form of inflammatory cardiomyopathy characterized by eosinophilic infiltration into myocardial tissue and subsequent release of substances with cell membrane damage and cell destruction. The degree of infiltration is thought to depend on the underlying condition, as well as the degree and duration of eosinophil exposure and ranges from mild localized disease to diffuse multifocal infiltrates associated with myocardial necrosis, thrombotic complications and endomyocardial fibrosis. The main causes of EM are hypersensitivity reactions, eosinophilic granulomatosis with polyangiitis, hypereosinophilic syndrome variants, infections and cancer. Clinical presentation can be variable, ranging from asymptomatic forms to life-threatening conditions, to chronic heart failure due to progression to chronic restrictive cardiomyopathy. Marked eosinophilia in peripheral blood, elevated serum eosinophilic cationic protein concentration and multimodality imaging may suggest the etiology of EM, but in most cases an endomyocardial biopsy must be performed to establish a definitive diagnosis. Systemic treatment varies greatly depending on the underlying cause, however the evidence of an eosinophilic infiltrate allows initiation of immunosuppressive therapy, which is the mainstay of treatment in idiopathic and in most forms of EM. Patients with helminthic infection benefit from anti-parasitic therapy, those with myeloid clone often need a tyrosine kinase inhibitor, while anticoagulant therapy should be undertaken in case of possible thrombotic complications.

Pediatric hypereosinophilic syndrome associated with liver damage, portal vein, splenic vein and superior mesenteric vein thromboses: a case report.

BACKGROUND: The hypereosinophilic syndrome (HES) is a group of rare blood disorders characterized by persistent eosinophilia and damage to multiple organs. HES can be either primary, secondary or idiopathic. Secondary HES are commonly caused by parasitic infections, allergic reactions or cancer. We described a pediatric case of HES associated with liver damage and multiple thrombi. A 12-year-old boy with eosinophilia was complicated with severe thrombocytopenia, liver damage, portal vein, splenic vein, and superior mesenteric vein thromboses. The thrombi recanalized after treatment with methylprednisolone succinate and low molecular weight heparin. No side effects appeared after 1-month. CONCLUSIONS: Corticosteroids should be used at an early stage of HES to prevent further damage to vital organs. Anticoagulants should be recommended only in cases with thrombosis which should be actively screened as a part of evaluation of end organ damage.

Hypereosinophilic syndrome with cutaneous manifestations successfully treated with Dupilumab as a sole agent: A case report.

Hypereosinophilic syndrome describes a process in which eosinophils in the peripheral blood are persistently increased, with variable clinical manifestations. Finding efficacious treatments for this disease can be challenging. This case describes a 72-year-old man with idiopathic hypereosinophilic syndrome with cutaneous manifestations who was successfully treated with dupilumab as a single agent therapy. There was complete clinical and biochemical resolution of disease (eosinophils levels decreased from 4.13 to 0.92) without complications.

Hypereosinophilic syndrome presenting with gastrointestinal manifestations.

Hypereosinophilic syndrome (HES) is a spectrum of diseases characterised by an elevated eosinophilic count causing end-organ damage. Differential diagnoses of hypereosinophilia are vast and include drug hypersensitivities, allergies, infections, cancers, autoimmune disorders and rare eosinophilic syndromes. Herein, we describe a case of a patient presenting with gastrointestinal (GI) symptoms including progressive dysphagia, abdominal distension, vomiting, diarrhoea and abdominal pain with significant peripheral eosinophilia who was found to have an overlap HES involving the GI tract. This patient's eosinophilia was rapidly corrected with intravenous methylprednisolone, and the patient experienced gradual resolution of clinical symptoms with maintenance oral prednisone. Due to the rarity and diverse presentation of HES, there are few large, longitudinal studies that describe disease progression and inform treatment guidelines. This case demonstrates the difficulty in designing a treatment regimen for these patients and emphasises the clinical need for improved understanding of HES.

Giant Coronary Artery Aneurysm with Thrombosis Complicated in a Patient with Idiopathic Hypereosinophilic Syndrome.

Idiopathic hypereosinophilic syndrome (iHES) is a rare systemic disease that is characterized by persistent peripheral eosinophilia (absolute eosinophil count ≥1500/uL) for more than 6 months, with end-organ damage and absence of a primary cause for eosinophilia. Coronary artery aneurysm (CAA) is a rare but life-threatening complication. Here, we report a case of CAA with thrombosis in a patient with iHES in whom the disease activity was well-controlled (eosinophil count <500/uL) for several years. Despite modest control of the disease activity, giant CAA can be associated with iHES; and therefore, close surveillance and monitoring for the development of complications is warranted.