Virtually any organ system may be involved, most frequently the heart, the central and peripheral nervous system, the lungs and the skin. Idiopathic hypereosinophilic syndrome complicated by central sinovenous thrombosis. Idiopathic hypereosinophilic syndrome associated pulmonary. Idiopathic hypereosinophilic syndrome and pregnancy. In addition to weight loss, anaemia, amenorrhoea, general lethargy. Sep 14, 2020 idiopathic hypereosinophilic syndrome ihes is one of numerous hypereosinophilic syndromes. Pdf the idiopathic hypereosinophilic syndrome researchgate. Idiopathic hypereosinophilic syndrome, as the name suggests, is a primary hypereosinophilia without an underlying cause that is characterized by persistent marked eosinophilia 1500 eosinophils. Background the hypereosinophilic syndrome is a group of diseases characterized by persistent blood eosinophilia, defined as more than 1500 cells per microliter with endorgan involvement and no rec. Neurological endorgan complications in hypereosinophilic syndrome are unusual and have been established to be of three types.
The differential diagnosis between idiopathic hypereosinophilic syndrome and other disorders known to course with vasculitis and hypereosinophilia is discussed. Idiopathic hypereosinophilic syndrome hes is a rare disease with no established effective therapy. It can be either primary or secondary, for example, due to parasitic infections or inflammation. Jun, 2017 idiopathic hypereosinophilic syndrome familial eosinophilia. Summary a 14 year old girl with idiopathic hypereosinophilic syndrome is described. In this reported case, the acute idiopathic hypereosinophilic syndrome was diagnosed in a 4year rottweiler female dog based on an increased eosinophilemia and the infiltration of stomach, small. How i treat hypereosinophilic syndromes blood american. Hypereosinophilic syndrome symptoms, diagnosis, treatment. Eosinophils can accumulate in multiple organs, most commonly involving the heart, skin, lungs, spleen, and liver. Hypereosinophilic syndrome hes is characterized by persistent hypereosinophilia. The idiopathic hypereosinophilic syndrome researchgate.
Idiopathic hypereosinophilic syndrome hes is a rare disorder characterized by peripheral eosinophilia exceeding 1500mm 3, a chronic course, absence of secondary causes, and signs and symptoms of eosinophilmediated tissue injury. Acute progressive stroke with middle cerebral artery. Idiopathic hypereosinophilic syndrome presenting with. In addition to weight loss, anaemia, amenorrhoea, general lethargy, anorexia, mouth ulcers, blisters of hands and feet, and petechial skin rash, she had features of involvement of the cardiovascular system as. Idiopathic hypereosinophilic syndrome ihes is characterized by the persistent elevation of blood eosinophils without any recognizable causes such as allergies, parasitic infections, drugs, connective tissue diseases, vasculitis, or malignancies. The patient presents with clinical manifestations of right ventricular systolic dysfunction resulted from severely increased ph. Oct 29, 2009 the idiopathic hypereosinophilic syndrome hes was first defined by chusid et al in 1975 as 1 persistent eosinophilia of 1. In this report, we describe the use of delayed, enhanced inversion recovery prepared viabil. Diagnosis requires marked peripheral eosinophilia and exclusion of infection, hematologic malignancy, and vasculitis. Idiopathic hypereosinophilic syndrome ihes is a leukoproliferative disorder and refers to a situation when there is an unexplained prolonged eosinophilia with associated organ system dysfunction. The idiopathic hypereosinophilic syndrome represents a leukoproliferative process likely caused by a number of disorders, all of which are marked by sustained overproduction of eosinophils in.
The syndrome is characterized by multiple organ involvement including the heart, nervous system, lungs, and gastrointestinal tract. Heterogeneity of human eosinophil glucocorticoid receptor expression in hypereosinophilic patients. Idiopathic hypereosinophilic syndrome is a rare disease which is diagnosed after excluding other conditions. Treatment of idiopathic hypereosinophilic syndrome with.
A, axial t2weighted mr image shows a hypointense left skull base mass arrows at the region of the left basisphenoid. Idiopathic hypereosinophilic syndrome ihes was diagnosed. Idiopathic hypereosinophilic syndrome with cutaneous. Who classification of myeloid disorders that overlap clinically with myeloproliferative hes pdf, 105. 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 14. A 46yearold woman with a history of idiopathic hypereosinophilic syndrome presented with multiple cranial nerve palsies. Keywords idiopathic hypereosinophilic syndrome, pulmonary hypertension, right heart catheterization.
Ct scan of a markedly enlarged spleen that has undergone total infarction in a patient with hes. Idiopathic hypereosinophilic syndrome, eosinophilic granulomatosis with polyangiitis formerly churgstrauss syndrome, eosinophilic gastrointestinal disorders allergic disorders, including asthma and atopic dermatitis, can be associated with he aec. The condition can affect several organ systems which includes. Almost no information about interactions of hyp ereosinophilia and pregnancy are available. It can cause irreversible organ dysfunction, disability, or death.
Occurring in idiopathic hypereosinophilic syndrome giovanni c. Hypereosinophilic syndrome is a disease characterized by a persistently elevated eosinophil count. Alemtuzumab therapy for hypereosinophilic syndrome and chronic eosinophilic leukemia. Hypereosinophilic syndrom hes is defined by showing eosinophilic infiltration in any tissue or organ and. The idiopathic hypereosinophilic syndrome represents a leukoproliferative process likely caused by a number of disorders, all of which are marked by. Thromboangiitis obliterans associated with idiopathic hypereosinophilia.
Idiopathic hypereosinophilic syndrome and chronic eosinophilic leukemia non. Infliximab therapy was initiated in late november 2005, with a sharp decrease in absolute eosinophil counts. Herein, we report on two cases of hypereosinophilic syndrome presenting as liver mass. Cardiac mri documented endomyocardial fibrosis complicated with biventricular thrombus. The idiopathic hypereosinophilic syndrome jama dermatology.
Hypereosinophilic syndrome an overview sciencedirect topics. Various neurological complications are reported, but very few have mentioned cns pathology and none has included cns vasculitis. J dermatol case rep 2014 4, pp 108114 idiopathic hypereosinophilic syndrome. Patients may ultimately progress to restrictive cardiomyopathy and valvular insufficiency because of. Normalization of eosinophil count was achieved after 3 monthly doses of infliximab, and the effect was maintained after 7 monthly doses. L present for longer than 6 months with evidence of organ injury related to the hypereosinophilia, such as cardiomyopathy, peripheral neuropathy, and encephalopathy. Diagnosis and novel approaches to the treatment of. Idiopathic hypereosinophilic syndromes and diagnostic. Idiopathic hypereosinophilic syndrome ihes is a rare myeloproliferative disease characterised by multisystem dysfunction and persistent, extreme eosinophilia of unknown cause.
Pdf the idiopathic hypereosinophilic syndrome represents a leukoproliferative process likely caused by a number of disorders, all of. Etiology, diagnosis, manifestations and management introduction hypereosinophilia is a clincial condition that can classified into secondary, clonal and idiopathic. Idiopathic hypereosinophilia is a rare disease that is characterized by hypereosinophilia in the peripheral circulation and damage to multiple organs, including the lungs, heart, skin, nervous system, and gastrointestinal tract. The second patient was a 54yearold man who presented with nausea and abdominal pain with significant weight loss. We report a pediatric case of ihes and the challenges to finding an effective treatment. I ntroduction idiopathic hypereosinophilic syndrome hes is a disorder characterized by a sustained eosinophilia of at least 6 months duration, multiple organ system infiltration and lack of evidence of known causes of eosinophilia.
The idiopathic hypereosinophilic syndrome represents a leukoproliferative process likely caused by a number of disorders, all of which are marked by sustained overproduction of eosinophils. Idiopathic hypereosinophilic syndrome hes was first defined by chusid et al in 1975 1 as unexplained eosinophilia of greater than 1500. Infliximab as a therapy for idiopathic hypereosinophilic. In 1968 hardyandandersondrewattention to the fact that persistent hypereosinophilia of any type could be associated with a rangeofsimilarcomplications and they grouped these together as the hypereosinophilic syndrome. Occasionally, the second form terminates as a blast cell leukaemia. A 14 year old girl with idiopathic hypereosinophilic syndrome is described. A 27yearold man presented with embolic encephalopathy. Elevated serum tryptase levels identify a subset of patients with a myeloproliferative variant of idiopathic hypereosinophilic syndrome associated with tissue fibrosis, poor prognosis and imatinib responsiveness. Eosinophil count in a patient with idiopathic hypereosinophilic syndrome. Elevated serum tryptase levels identify a subset of. Idiopathic hypereosinophilic syndrome with skull base. How i treat hypereosinophilic syndromes blood american society.
Cardiopulmonary and gastrointestinal manifestations of. Idiopathic hypereosinophilic syndrome hes is a rare entity of unknown etiology with a prognosis strongly related to heart involvement that may lead to restrictive endomyocardi opathy 1. Treatment of the primary disease will usually resolve the eosinophilia. Diagnosis of idiopathic hypereosinophilic syndrome hes can be retained after elimination of parasitic, allergic and haematological causes in the presence of a blood eosinophilia greater than 1. Ihes is a leukoproliferative disorder causing multiorgan damage.
Rare eosinophilic disorders, idiopathic hypereosinophilic syndrome. Reactive eosinophilia, chronic eosinophilic leukemia and. Moderate to severe eosinophilia may be caused by the idiopathic hypereosinophilic syndrome hes, but the com monest cause of eosinophilia worldwide is. Idiopathic hypereosinophilic syndrome with multiple organ. Idiopathic hypereosinophilic syndrome with skull case. Pdf idiopathic hypereosinophilic syndrome in a rottweiler. Idiopathic hypereosinophilic syndrome hes is empirically defined as the presence of prolonged eosinophilia without an identifiable underlying cause, and with evidence of endorgan dysfunction. Idiopathic hypereosinophilic syndrome with pulmonary. Idiopathic hypereosinophilic syndrome presenting with severe. An unusual presentation of idiopathic hypereosinophilic. Oct 01, 2020 background idiopathic hypereosinophilic syndrome ihes is associated with various organ system dysfunctions. Cerebral infarction secondary to occlusion of large cerebral artery is rarely reported.
The idiopathic hypereosinophilic syndrome 603 tures of a leukaemic disease, characterised as eosinophilic leukaemia. Idiopathic hypereosinophilic syndrome presenting with liver. Idiopathic hypereosinophilic syndrome with 20 years of. Acute aortic thrombosis despite anticoagulant therapy in idiopathic hypereosinophilic syndrome. Idiopathic hypereosinophilic syndrome radiology reference. The eosinophilias, including the idiopathic hypereosinophilic. Hypereosinophilic syndrome hes describes a heterogeneous. Hypereosinophilic syndrome hes is an uncommon syndrome characterized by peripheral blood eosinophils count of more than 1,500mm3 with associated tissue damage. Idiopathic hypereosinophilic syndrome with skull case report.
Article text new page beta english pdf article in xml format how to cite this article scielo analytics automatic translation. Hypereosinophilic syndrome genetic and rare diseases. Hypereosinophilic syndrome hes is characterized by persistent. An unusual presentation of idiopathic hypereosinophilic syndrome. Hypereosinophilia is the term applied empirically when the blood. New agents, such as imatinib mesylate and mepolizumab, target specific abnormalities associated with hes and have the potential to improve clinical outcomes in hes. Idiopathic hypereosinophilic syndrome presenting as.
Here we described a patient with ihes presented progressive multiple cerebral infarctions caused by bilateral middle cerebral. We present a case of a 49yearold asian female with recurrent hospital admissions for cholecystitis, gastritis, urinary cystitis, and. It has been reported that interleukin5 il5 produced by helper t cells plays a major role in. Idiopathic hypereosinophilic syndrome associated with rapid. Idiopathic hypereosinophilic syndrome presenting as iga. Salanitri1 diopathic hypereosinophilic syndrome ihes is characterized by peripheral blood eosinophilia and multiorgan dysfunction in the absence of known causes of hypereosinophilia. Molecular pathogenesis and treatment perspectives for. Idiopathic hypereosinophilic syndrome in a rottweiler. Eosinophilic heart disease is another manifestation of ead. Idiopathic, na, varied signs and symptoms, may affect any organ system.
Orphanet is a european reference portal for information on rare diseases and orphan drugs. Eosinophilic myocarditis is a rare form of myocarditis. Idiopathic hypereosinophilic syndrome associated pulmonary hypertension in a child. Abstract idiopathic hypereosinophilic syndrome ihes is one of numerous hypereosinophilic syndromes. The onset of symptoms is insidious in most of the cases and. Despite renal pathology in idiopathic hypereosinophilic syndrome being extremely rare, in recent years some cases have appeared describing different types of renal involvement. Hypereosinophilic syndrome symptoms and causes mayo clinic. The involvement of the pulmonary artery in patients with idiopathic hypereosinophilic syndrome is an uncommon finding.
Herein, we report a case of hypereosinophilic syndrome diagnosed as chronic myeloid leukemia with eosinophilia. Endomyocardial fibrosis and intracardiac thrombus occurring. We hereby report a female patient who presented with sudden onset upper. The diagnosis must be made in time in order to initiate treatment promptly and obtain a complete recovery in renal function. The patient described here was responsive to prednisolone and thalidomide. Neurologic abnormalities have been previously noted in this syndrome.
Hypereosinophilic syndrome is a term coined in 1968 to refer to a heterogeneous group of diseases characterized by persistent eosinophilia of unknown origin complicated by end organ damage. Idiopathic hypereosinophilic syndrome ihes is a rare disorder. The idiopathic hypereosinophilic syndrome archives of disease in. We read with interest the case report by fujii et al. Idiopathic hypereosinophilic syndrome hes is characterized by an absolute eosinophil count greater than 1500 cellsmm3 observed at least twice with a minimum interval of 4 weeks, multiorgan involvement, and presence of tissue damage without an identifiable underlying cause 1. This article has been cited byother articles in pmc. Hypereosinophilic syndromes hess are a group of rare disorders. In addition to weight loss, anaemia, amenorrhoea, general lethargy, anorexia, mouth ulcers, blisters of hands and feet, and petechial skin rash, she had features of involvement of the. Idiopathic hypereosinophilic syndrome journalagent.
Idiopathic hypereosinophilic syndrome with 20 years of diagnostic. Multimodality imaging approach1 eosinophilassociated diseases eads are a diverse group of disorders characterized by an increase in circulating or tissue eosinophils. There are some discrepancies in this case report that should be noted and require further clarification. Hes cases will be classified as idiopathic table 2. Treatment of patients with the hypereosinophilic syndrome. Idiopathic hypereosinophilic syndrome with pulmonary hypertension. In addition to weight loss, anaemia, amenorrhoea, general lethargy, anorexia, mouth ulcers, blisters of hands and feet, and petechial skin rash, she had features of involvement of the cardiovascular system as the major complication. It is characterised by persistent eosinophilia with eosinophil mediated tissue infiltration and organ dysfunction. After a comprehensive search of the published reports. Here we present a 42yearold patient complaining of moderate to severe chest pain and shortness of breath, and typical ischaemic electrocardiography changes were recorded. Clinical features of ihes vary widely, as it may present with dermatological, pulmonary, gastrointestinal, cardiac or neurological symptoms. 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 14, with no obvious cause for eosinophilia. One patient was a 22yearold woman presented with fever, upper abdominal pain, nauseavomiting and a mass in the right liver lobe.
The incidence of ihes among children is unknown, but it is considered a rare disease. Hypereosinophilic hypereeosinophilik syndrome hes is a group of blood disorders that occur when you have high numbers of eosinophils white blood cells that play an important role in your immune system. Department of pathology, nyu langone medical center, new york, new york. Review an unusual presentation of idiopathic hypereosinophilic syndrome. Cardiopulmonary and gastrointestinal system involvement can be due to primary ead with no known cause or can be sec. Hypereosinophilic syndrome hes is a rare but lifethreatening multiorgan disease which can be complicated by stroke, with devastating outcomes. Infliximab as a therapy for idiopathic hypereosinophilic syndrome. A representative example of stroke syndrome due to idiopathic hypereosinophilic syndrome ihes is herein presented along with the other 21 cases published. Hypereosinophilic syndrome an overview sciencedirect. Secondary hypereosinophilia is caused by tissue helminthic infection, parasitic infestations, atopy, allergic reactions, vasculitis, or drugs. Idiopathic hypereosinophilic syndrome in hemodialysis. Sinus thrombosis in idiopathic hypereosinophilic syndrome causing fatal cerebral haemorrhage. Idiopathic hypereosinophilic syndrome chest journal american. Pubmed is a searchable database of medical literature and lists journal articles that discuss hypereosinophilic syndrome.
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