Pulmonary thromboembolism in a patient with idiopathic hypereosinophilic syndrome

Abstract

We report a successful treatment in a patient with idiopathic hypereosinophilic syndrome (HES) presenting with left pulmonary truncal thromboembolism and right pleural effusion. A treatment with urokinase infusion by a Swan-Gants catheter near a left pulmonary thrombus was performed and left pulmonary arterial occlusions were recanalized completely. After corticosteroid therapy was started, right pleural effusion disappeared. Both anticoagulant and corticosteroid therapy was necessary for treatment in HES patients who had thromboembolic episodes.     

Eosinophilic cholangiopathy with concurrent eosinophilic colitis in a patient with idiopathic hypereosinophilic syndrome

Eosinophilic cholangiopathy is a rare cholestatic disorder of unknown etiology. Simultaneous histologic documentation of eosinophilic involvement of the bile ducts and gastrointestinal tract has been reported previously in only a few well-documented cases. We report a 52-year-old man with a history of idiopathic hypereosinophilic syndrome who presented with acute diarrhea and cholestatic hepatitis. Colonoscopy revealed colitis, and a biopsy showed eosinophilic infiltrates in the colonic mucosa. Liver biopsy showed dense eosinophilic inflammation in the portal areas with bile duct damage. This case demonstrates the entity of hepatic involvement in idiopathic hypereosinophilic syndrome and the difficulties related to diagnosis and treatment.     

Heart failure mimicking prior myocardial infarction in a patient with idiopathic hypereosinophilic syndrome

Idiopathic hypereosinophilic syndrome (IHES) is characterized by sustained, nonreactive hypereosinophilia with eosinophilia-associated organ damage. Cardiac involvement occurs in about 60% of patients with HES and it is the major cause of mortality in these patients. Cardiac dysfunction is reversible only after early corticosteroid (CS) initiation.Herein we report a 33-year old male who was referred to our Cardiology Department with electrocardiographic and echocardiographic abnormalities suggesting myocardial infarction. At presentation he complained of dyspnea, cough and persistent fever. His white blood cell (WBC) count was elevated, with eosinophil predominance in the differential. After cardiological and haematological work-up, the final diagnosis of HES-associated cardiac involvement was established. Early treatment with CS led to eosinophil count normalization with only moderate cardiac function improvement. Currently, the patient is in good condition overall and is in NYHA class II while still on prednisone.     

Arthritis in idiopathic hypereosinophilic syndrome

A 54-year-old man was admitted for tenosynovitis of the wrists, olecranon bursitis, stiffness, progressive flexion of the fingers, subcutaneous nodules, and persistent blood eosinophilia. A high eosinophil count was detected in his synovial fluid. We review the literature on idiopathic hypereosinophilic syndrome. This case provides evidence that nonerosive polyarthritis with periarticular involvement and nodules can be manifestations of this syndrome.     

Intracardiac echo guided valvuloplasty of a stenotic tricuspid prosthetic valve in a patient with idiopathic hypereosinophilic syndrome

A 52-year-old male with idiopathic hypereosinophilic syndrome (HES) was transferred to our institution following the development of acute respiratory failure and shock. He had previously undergone tricuspid valve replacement with bioprosthetic valves on two occasions: the initial surgery for severe native tricuspid valve stenosis and the redo surgery for severe prosthetic valve stenosis and regurgitation. Conventional imaging assessment using transoesophageal echocardiography was suboptimal and comprehensive assessment of prosthetic valve function was aided by the use of intracardiac echocardiography (ICE). ICE provided high quality 2D imaging of the prosthesis demonstrating thrombus-like material coating the inner surfaces of the prosthetic valve stents effectively forming a tunnel-like obstruction. Unusual hemodynamics secondary to severe tricuspid stenosis were demonstrated by CW Doppler with intermittent "signal fusion" resulting from blunted respiratory variation in the markedly elevated right atrial pressure relative to right ventricular pressure. Successful balloon valvuloplasty was performed with ICE proving highly valuable in guiding balloon position as well as monitoring the efficacy of the subsequent inflations.     

The idiopathic hypereosinophilic syndrome.

Idiopathic hypereosinophilic syndrome (IHES) is characterized by sustained (> 6 months) peripheral eosinophilia (> 1500 cells/mm3), organ damage, and exclusion of other causes. IHES is clinically and pathogenetically heterogenous, and several potential disease mechanisms have been described. The prognosis for patients with IHES has improved with targeted therapies and aggressive management of complications. We report a case of IHES and review the clinical features, evaluation, and options for treatment.     

Allogeneic bone marrow transplantation in a patient with hypereosinophilic syndrome

We describe a 32-year-old man with idiopathic hypereosinophilic syndrome (HES) who presented with pulmonary dysfunction, thrombocytopenia, lymphadenopathy, and hepatosplenomegaly. The patient developed progressive disease on prednisone and hydroxyurea therapy, and he underwent a successful allogeneic bone marrow transplantation (BMT). The patient is asymptomatic with no evidence of eosinophilia 30 months after transplantation. There is currently no cure for patients with HES, and BMT should be considered in selected patients. © 1996 Wiley-Liss, Inc.     

Mitral valve repair in idiopathic hypereosinophilic syndrome

Idiopathic hypereosinophilic syndrome is a rare systemic disease than can cause multiple organ failure by eosinophilic infiltration. Cardiac involvement is characterized by endocardial fibrosis and overlying thrombus, leading to restrictive cardiomyopathy and valvular dysfunction. Surgical experience of patients with mitral dysfunction caused by this syndrome is limited, and valvular replacement is most often performed. Mechanical valvular replacement has a high incidence of recurrent obstructive thrombosis, and replacement with a bioprosthesis is recommended, despite associated restrictive cardiomyopathy. A patient is described who presented with mitral insufficiency associated with idiopathic hypereosinophilic syndrome, and underwent mitral valve repair.     

Neurologic dysfunction in the idiopathic hypereosinophilic syndrome

Three specific patterns of neurologic deficit were seen in a group of 52 patients with the idiopathic hypereosinophilic syndrome. Central nervous system dysfunction was seen in 7 patients (15%), 4 of whom had a distinctive encephalopathy characterized by behavioral disturbances and upper motor neuron signs. Peripheral neuropathy was found in 27 patients (52%), with a sensory polyneuropathy the commonest form (in 23 patients), although mononeuritis multiplex and radiculopathy were also seen. Central nervous system abnormalities from embolic disorders were seen in 6 patients (12%). Thus, a total of 65% of these patients had some neurologic dysfunction. Although the spectrum of neurologic disease is broad and includes very different manifestations, we conclude that distinct patterns of neurologic involvement are characteristic of this syndrome.     

Hepatic vein obstruction in idiopathic hypereosinophilic syndrome

We report an association between idiopathic hypereosinophilic syndrome and obstruction of the hepatic veins (Budd-Chiari syndrome). Budd-Chiari syndrome was assessed by liver biopsy and hepatic phlebography and documented by computed tomography. Postmortem examination revealed fibrous occlusion of the hepatic venous tree, as well as fibrosis of the endocardium and of myocardial and pulmonary vessels. To our knowledge, the association between idiopathic hypereosinophilic syndrome and Budd-Chiari syndrome has never previously been reported. Since it has been suggested that hypereosinophilia might cause endothelium damage, a link between these two entities is postulated.     

Cardiac manifestations of idiopathic hypereosinophilic syndrome

BACK GROUND: The aim of our study is to describe the more common cardiac manifestations of idiopathic hypereosinophilic syndrome representing the major cause of mortality. MAIN POINTS: Current therapy consists of corticosteroid, hydroxyurea and interferon alpha. Recent publications confirm the activity of imatinib mesylate, a selective tyrosine kinase inhibitor, in patients with idiopathic hypereosinophilic syndrome. In cases with marked valvular compromise or with endomyocardial thrombosis or fibrosis, cardiac surgery can provide substantial benefits. PERSPECTIVES: A better understanding of the pathophysiology of this syndrome could lead to the development of new therapeutic agents.