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 recognized. Hypereosinophilic syndrome hematology and oncology. The diagnostic criteria were later introduced in 1975 by chusid. List of hypereosinophilic syndrome medications 2 compared. Incidence of myeloproliferative hypereosinophilic syndrome in the united states and an estimate of all hypereosinophilic syndrome incidence. Although the existence of clinical subtypes or variants of hes has been appreciated for some time, the recent characterization of some of these variants at the molecular and immunologic levels has. Recent evidence suggests at least two distinct underlying hematological disorders involving myeloid and lymphoid cells, respectively. One of the observed limitations of this definition is the requirement of elevated eosinophil levels 1. Peripheral eosinophilia with tissue damage has been noted for approximately 80 years, but hardy and anderson first described the specific syndrome in 1968. The hypereosinophilic syndromes annals of internal medicine.
Orphanet is a european reference portal for information on rare diseases and orphan drugs. Signs and symptoms vary significantly based on which parts of the body are affected. Allergies, asthma, parasitic infections, and certain medicines may increase the number of eosinophils. Mar, 2018 hypereosinophilic syndrome is a disease that is characterized by extremely high blood eosinophils. According to the surveillance, epidemiology and end results database, the estimated morbidity is between 0. Hypereosinophilic syndrome genetic and rare diseases. More than half of all patients have cutaneous involvement. Aug 21, 2019 hypereosinophilic syndrome hes encompasses a wide range of clinical manifestations sharing 3 features defined by chusid et al.
Sep 19, 2014 hypereosinophilic syndrome hes is currently defined as a blood eosinophil count 1. Hypereosinophilic syndromes are a group of disorders characterised by significant eosinophilia and organ damage. Although any organ system can be involved in hes, the heart, central nervous system, skin, and respiratory tract are the most. Idiopathic hypereosinophilic syndrome ihes is a group of. In a minority of the reported cases, skin involvement has been the only manifestation of hes. Hypereosinophilic syndromes hess are a group of rare disorders characterized by peripheral blood eosinophilia of 1. Jan 27, 2015 incidence of myeloproliferative hypereosinophilic syndrome in the united states and an estimate of all hypereosinophilic syndrome incidence. Hypereosinophilic syndrome hes is characterized by peripheral blood eosinophilia 1. They have proven challenging to define, diagnose, and study for many years, due in part to their variable clinical presentations, the overlap between neoplastic and reactive eosinophilia, and the lack of a universal marker of eosinophil clonality. Over time, the excess eosinophils enter various tissues, eventually damaging your organs.
Natural history of hypereosinophilia and hypereosinophilic syndromes cohesion the safety and scientific validity of this study is the responsibility of the study sponsor and investigators. The presentation can be sudden and dramatic, with cardiac, neurologic, or thrombotic complications, but, more often, the onset is insidious. Hypereosinophilic syndrome hes is a heterogeneous group of disorders, first described in 1968 by hardy and anderson. The hypereosinophilic syndrome and leukapheresis annals of. Hess are pleomorphic in clinical presentation and can be idiopathic or associated with a variety of underlying conditions. Hypereosinophilic syndrome clinical trials mayo clinic. Hypereosinophilic syndrome with eosinophilic gastritis.
The terms hypereosinophilic syndromes returned 6 free, fulltext research articles. Hypereosinophilic syndrome consists of a group of disorders characterized by abnormal accumulation of eosinophils in the blood or peripheral tissues, independent of known secondary causes of eosinophilia such as parasitic infection. This means that the body manufactures too many white blood cells in the bone marrow. Although the existence of clinical subtypes or variants of hes has been appreciated for some time, the recent characterization of some of these variants at the molecular and immunologic levels has demonstrated. Hypereosinophilic syndromes orphanet journal of rare. Jun 20, 2017 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. It occurs when the number of eosinophils rises to more than 1500 per cubic millimeter of blood. Diagnosis and novel approaches to the treatment of. Rothenberg and colleagues march 20 issue1 provide data on the use of mepolizumab, an antiinterleukin5 monoclonal antibody, for corticosteroid sparing in patients with non. Hypereosinophilic syndrome usually presents with fever, weight loss, fatigue, and rash. Although any organ may be involved, the heart, lungs, spleen, skin, and nervous system are typically affected.
Pdf eosinophilia and hypereosinophilic disorders update on. For severe drug reactions, including dress syndrome, adjunct treatment with steroids may be helpful 16. Hypereosinophilic syndrome is rare, has an unknown prevalence, and most often affects people age 20 through 50. Hypereosinophilic syndrome article about hypereosinophilic. Dermatologic manifestations of hypereosinophilic syndrome. How i diagnose hypereosinophilic syndromes european. Pathologic conditions accompanied by blood eosinophilia are quite frequent.
Prognostic risk evaluation for hypereosinophilic syndrome hes and idiopathic hypereosinophilia ihe and the role of nextgeneration sequencing rochester, mn the purpose of this study is to. Benralizumab for pdgfranegative hypereosinophilic syndrome. These aberrant lymphocytes function abnormally by stimulating the proliferation and maturation of. Hypereosinophilic hypereeosinofillick syndrome, also known as hes, is a rare blood disease in which the body produces too much of a particular type of blood cell called eosinophils eeo.
Hypereosinophilic syndrome is a group of related conditions varying from a localised benign form loeffler syndrome, to a disseminated form producing a marked eosinophilic leukocytosis and organ dysfunction as a result of infiltration by mature eosinophils. Hypereosinophilic syndrome hes is a type of rare blood disorder. Hypereosinophilic syndrome symptoms and causes mayo clinic. The three diagnostic criteria for this disorder are 1 eosinophilia of greater. Hypereosinophilic syndrome hes is a myeloproliferative disorder mpd characterized by persistent eosinophilia that is associated with damage to multiple organs. When innocent heroworship goes unrequited, the consequences can be dire for both the admirer and the admired. Nov 22, 2012 hypereosinophilic syndrome hes is a type of rare blood disorder. Hypereosinophilic syndrome has the potential to disrupt every organ in the body and, although it more frequently presents insidiously, sudden and acute deterioration is not uncommon and affects mostly the nervous and cardiovascular system. The hypereosinophilic syndrome is a group of diseases characterized by persistent blood eosinophilia, defined as more than 1500 cells per microliter with end. Hypereosinophilic syndromes are a heterogeneous group of uncommon disorders. Rothenberg and colleagues march 20 issue1 provide data on the use of mepolizumab, an antiinterleukin5 monoclonal antibody, for. Cardiac dysfunction occurs frequently in all forms of hes and is a major cause of morbidity and mortality. In 1975, chusid et al 2 described 14 cases of idiopathic hes defined by the presence of an aec.
The condition can affect several organ systems which includes. A heterogeneous group of disorders with the common feature of prolonged eosinophilia of unknown cause and associated organ system dysfunction, including the heart, central nervous system, kidneys, lungs, gastrointestinal tract, and skin. Although any organ system can be involved in hes, the heart, central nervous system, skin, and respiratory tract are the most commonly affected. It occurs when an individuals blood has very high numbers of eosinophils. A group of symptoms that collectively indicate or characterize a disease, disorder, or other condition considered abnormal. It is characterized by a constantly elevated eosinophil count in blood for a minimum of 6 months without any identifiable cause.
Hypereosinophilic syndrome symptoms, diagnosis, treatment. Intraventricular thrombus progression due to poor compliance with anticoagulant treatment in a patient with hypereosinophilic and antiphospholipid syndromes. Hypereosinophilic syndrome idiopathic hypereosinophilic. Listing a study does not mean it has been evaluated by the u. Hypereosinophilic syndrome hes is a heterogeneous group of disorders characterized by sustained overproduction of eosinophils. Hypereosinophilic syndrome is a heterogeneous group of rare disorders characterized by marked blood or tissue eosinophilia resulting in a wide variety of clinical manifestations.
Patients receiving prompt diagnosis and treatment have very good prognoses. Heshypereosinophilic syndrome ihesidiopathic hypereosinophilic syndrome lheslymphocytic variant hes mhesmyeloid hes mnmyeloid neoplasm pdgfraplateletderived growth factor a sohessingleorgan hes the end organ manifestations of hes are extremely varied, ranging from bothersome symptoms, such as urticaria, myalgia. Hypereosinophilic syndrome hes is a group of conditions that causes you to have too many eosinophils. Hypereosinophilic syndrome hematology and oncology msd. Due to the large collection of white blood cells in the bone marrow, they start to spill out of the bone marrow and start accumulating in other tissues and in the blood. The idiopathic hypereosinophilic syndrome article pdf available in archives of dermatology 25. Causes of this blood disorder have yet to be discovered. Hypereosinophilic syndrome causes, symptoms, diagnosis. Cardiac damage is the major cause of death and pathogenesis.
Hypereosinophilic syndrome hes refers to a rare group of conditions that are associated with persistent eosinophilia with evidence of organ involvement. To emphasize that such patients are part of a continuum, we propose the term hypereosinophilic syndromes to encompass the entire group. What is the prognosis of hypereosinophilic syndrome. These white blood cells then attack organ systems like the skin, lungs, brainnerves and heart. Interferon alpha prevents spontaneous apoptosis of clonal th2 cells associated with chronic hypereosinophilia. Hypereosinophilic syndrome presenting with multiple organ. 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. Clinical manifestations of the condition are highly variable, ranging from asymptomatic eosinophilia to severe. 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. What is hypereosinophilic syndrometypessymptomstreatment. Hypereosinophilic syndrome is a leukoproliferative disease characterised by sustained overproduction of eosinophils. Hypereosinophilic syndrome definition of hypereosinophilic. We therefore suggest that the term idiopathic should be abandoned in the classification of hes. It is a rare, multisystem, heterogeneous syndrome with significant mortality.
Most commonly, red swollen itchy nodules lumps have been described. Pixars computeranimated film the incredibles 2004 presents a case in point in one buddy pine, a decidedly nonsuperpowered adolescent with a talent for invention and a stalkerlike obsession with the ultrastrong superhero known as mr. There is a massive increase in the number of eosinophils in the blood. Hypereosinophilic syndrome is a disease that is characterized by extremely high blood eosinophils. The syndrome is characterized by persistent peripheral eosinophilia of 1500 cellsmicroliter, evidence of end organ damage by eosinophil infiltration, and no.
L for 6 months or death before that time, the absence of a known. Hes can also be discovered incidentally in up to 12% of patients 11. Hypereosinophilic syndromes hes constitute a rare and heterogeneous group of disorders, defined as persistent and marked blood eosinophilia 1. Practical approach to the patient with hypereosinophilia ncbi. Although these disorders had long been considered idiopathic eg, as in the idiopathic hypereosinophilic syndrome, ihes, etiologies for some forms of hes have been described. Hypereosinophilic syndrome is a disease characterized by a persistently elevated eosinophil count. Imatinib treatment significantly decreased leukemic growth and. Jul 17, 2018 hypereosinophilic syndrome is a heterogeneous disease process. Treatment of hypereosinophilic syndrome in a child using cyclosporine. Guideline for the investigation and management of eosinophilia. After evaluation of secondary causes of eosinophilia, the 2008 world health.
There is a massive increase in the number of eosinophils in the blood, mimicking leukaemia, and. Idiopathic hypereosinophilic syndrome presenting with multip. Natural history of hypereosinophilia and hypereosinophilic. Symptoms of hypereosinophilic syndrome mock those of other medical conditions, making it difficult for. The lymphoid variant of hypereosinophilic syndrome. The term hypereosinophilic syndrome hes is used to describe conditions. Symptoms vary, depending on the organ involved, and may include pruritic skin ulcers or erythroderma, endomyocarditis, lymph. To identify predictors of inferior overall survival in hesihe patients including molecular parameters using nextgeneration sequencing performed at the. However, changes to this definition have been proposed based on recent studies identifying.
Pdf eosinophils are innate immune leukocites recruited in large numbers to sites of allergic inflammation and parasitic infections. Hypereosinophilic hypereeosinofillick syndrome hes is a group of rare blood disorders. An eosinophil is a type of white blood cell that plays an important role in the immune system. Hypereosinophilic syndrome an overview sciencedirect topics. Hypereosinophilic syndrome an overview sciencedirect. Only some patients with prolonged eosinophilia develop organ dysfunction that characterizes hypereosinophilic syndrome. The hypereosinophilic syndromes annals of internal. Lymphocytevariant hypereosinophila, is a rare disorder in which eosinophilia or hypereosinophilia i. This may or may not be associated with tissue eosinophilia as confirmed by biopsy 3. A rash is present in over 50% of patients, but is nonspecific in appearance.
Jun, 2017 idiopathic hypereosinophilic syndrome familial eosinophilia. Hypereosinophilic syndrome is a rare blood disease that causes an overproduction of eosinophil, or white blood cells, which can damage tissues and organs, such as the heart and liver. The term hypereosinophilic syndrome was first coined in 1968 by hardy and anderson. Hypereosinophilic syndrome hes encompasses a group of leukoproliferative disorders with variable involvement of the internal organs.
The treatment of these disorders is discussed separately, as is the initial approach. Idiopathic hypereosinophilic syndrome hes is a rare disorder characterized by marked peripheral blood eosinophilia in association with eosinophilic tissue infiltrate and ensuing damage andor dysfunction in a setting where underlying diseases known to cause hypereosinophilia have been excluded adequately. Hypereosinophilic syndromes hes are types of disorders that happen when eosinophils white blood cells are overproduced by a persons body. Hypereosinophilic syndromes hes are a collection of disorders defined by persistent and significantly elevated levels of eosinophils in the blood, in addition to evidence of direct damage to at least one organ. Dec 31, 2014 idiopathic hypereosinophilic syndrome hes is a rare disorder characterized by marked peripheral blood eosinophilia in association with eosinophilic tissue infiltrate and ensuing damage andor dysfunction in a setting where underlying diseases known to cause hypereosinophilia have been excluded adequately. Clinical and biological features of patients with the idiopathic hypereosinophilic syndrome hes are heterogeneous. Patients requiring emergency treatment for severe or life. Hypereosinophilic syndrome hematology and oncology merck. It should be noted that, in some cases, eosinophilia. Since the first description of eosinophilic endomyocardial fibrosis by loeffler in 1934, 1 the definition and classification of hypereosinophilic syndromes hess have evolved considerably. How i treat hypereosinophilic syndromes blood american.
Hypereosinophilic syndrome hes is a heterogeneous group of conditions that is defined at its core by hypereosinophilia he blood eosinophil count of 1. Hypereosinophilic syndrome hes encompasses a diverse group of disorders characterized by serum eosinophil levels of 1. The increase in the eosinophilic count should not be a result of other definitive causes of hypereosinophilia, such as allergic reactions or parasitic infections. Alemtuzumab therapy for refractory idiopathic hypereosinophilic syndrome with abnormal t cells. Pubmed is a searchable database of medical literature and lists journal articles that discuss hypereosinophilic syndrome.