Hypereosinophilic Syndrome (HES) is a myeloproliferative disorder with persistent eosinophilia to cause organ damage.
Idiopathic HES diagnosis requires sustained AEC above 1500/µL for >6 months with tissue damage.
Hardy and Anderson described eosinophilia and tissue damage syndrome in 1968 after 80 years.
Genetic eosinophilia has stable eosinophil levels and mild symptoms. Overactive eosinophils cause tissue damage, inflammation, fibrosis, and organ malfunction.
HES is classified as:
Myeloproliferative
Lymphocytic
Idiopathic
Familial
Secondary eosinophilia is a cytokine-derived reactive phenomenon. Clonal eosinophilia is diagnosed using bone marrow histology and molecular genetics.
Idiopathic eosinophilia excludes secondary and clonal causes of elevated eosinophils. Autonomous proliferation causes chronic eosinophilic leukemia.
Epidemiology
Idiopathic form is rarely noticed. In the US, allergies are the main cause of eosinophilia, while parasitosis is more common globally.
No racial predilection for HES syndrome while it has 9:1 male predominance ratio. Peak incidence shows in patients between 20 to 50 years old.
HES is rare in children and its incidence decreases in the elderly population.
Anatomy
Pathophysiology
Eosinophils survive in tissues for weeks with sustained cytokine presence. Only eosinophils, basophils, and precursors have specific receptors.
Eosinophils die within 48 hours without cytokines due to toxic cationic proteins in granules. Toxins include major basic protein and eosinophil-derived substances.
Eosinophilic peroxidase and respiratory burst lead to free radical production damaging tissues. HES syndrome causes organ damage from eosinophil infiltration and mediator release in tissues.
Etiology
The causes of HES are:
Genetic Mutations
Cytokine Dysregulation
Immune System Dysfunction
Environmental and Infectious Triggers
Genetics
Prognostic Factors
It has an unpredictable outcome and can be deadly without treatment.
Idiopathic hypereosinophilic syndrome is generally mild, but patients with certain characteristics or heart failure fare worse.
Poor outcomes seen in HES with myeloproliferative features and leukocytosis over 90,000/ÎĽ L.
The syndrome has diverse complications based on affected organ systems during development.
Clinical History
Collect details including presenting symptoms, family and medical history to understand clinical history of patient.
In the initial treatment phase, the goal is to manage eosinophil levels and stabilize the patient’s vital signs.
Pharmacologic therapy is effective in the treatment phase as it includes use of corticosteroids, antineoplastic agents, immunomodulators, and interleukin inhibitors.
In supportive care and management phase, patients should receive required attention such as lifestyle modification and surgical intervention.
The regular follow-up visits with the physician are scheduled to check the improvement of patients along with treatment response.
Hypereosinophilic Syndrome (HES) is a myeloproliferative disorder with persistent eosinophilia to cause organ damage.
Idiopathic HES diagnosis requires sustained AEC above 1500/µL for >6 months with tissue damage.
Hardy and Anderson described eosinophilia and tissue damage syndrome in 1968 after 80 years.
Genetic eosinophilia has stable eosinophil levels and mild symptoms. Overactive eosinophils cause tissue damage, inflammation, fibrosis, and organ malfunction.
HES is classified as:
Myeloproliferative
Lymphocytic
Idiopathic
Familial
Secondary eosinophilia is a cytokine-derived reactive phenomenon. Clonal eosinophilia is diagnosed using bone marrow histology and molecular genetics.
Idiopathic eosinophilia excludes secondary and clonal causes of elevated eosinophils. Autonomous proliferation causes chronic eosinophilic leukemia.
Idiopathic form is rarely noticed. In the US, allergies are the main cause of eosinophilia, while parasitosis is more common globally.
No racial predilection for HES syndrome while it has 9:1 male predominance ratio. Peak incidence shows in patients between 20 to 50 years old.
HES is rare in children and its incidence decreases in the elderly population.
Eosinophils survive in tissues for weeks with sustained cytokine presence. Only eosinophils, basophils, and precursors have specific receptors.
Eosinophils die within 48 hours without cytokines due to toxic cationic proteins in granules. Toxins include major basic protein and eosinophil-derived substances.
Eosinophilic peroxidase and respiratory burst lead to free radical production damaging tissues. HES syndrome causes organ damage from eosinophil infiltration and mediator release in tissues.
The causes of HES are:
Genetic Mutations
Cytokine Dysregulation
Immune System Dysfunction
Environmental and Infectious Triggers
It has an unpredictable outcome and can be deadly without treatment.
Idiopathic hypereosinophilic syndrome is generally mild, but patients with certain characteristics or heart failure fare worse.
Poor outcomes seen in HES with myeloproliferative features and leukocytosis over 90,000/ÎĽ L.
The syndrome has diverse complications based on affected organ systems during development.
Collect details including presenting symptoms, family and medical history to understand clinical history of patient.
Persistent urticaria, eczema, pruritus, gradual development of fatigue, peripheral edema, and dyspnea
Angiolymphoid hyperplasia
Atopic dermatitisc
Eosinophilic toxocariasis
Collagen vascular diseases
Not recommended to treat asymptomatic cases for hypereosinophilic syndrome due to high risks.
Patients are monitored with serum troponin every 3 to 6 months, for echocardiography and pulmonary tests every 6 to 12 months.
Initial treatment for patients without FIP1L1/PDGFRA mutation is glucocorticoids.
In unresponsive cases, use interferon alpha and hydroxyurea as second-line drugs.
Mepolizumab reduces flares in FIP1L1/PDGFRA-negative hypereosinophilic syndrome with interleukin-5 specificity.
Recurrent thromboembolic events happen in hypereosinophilic syndrome despite anticoagulant therapy.
Emergency leukapheresis in HES indicated to control symptoms from hyperleukocytosis.
Hematology
Use HEPA filters to reduce allergens and dehumidifiers to reduce mold growth.
Ensure proper ventilation in kitchens and bathrooms to prevent the humidity.
Follow good hand hygiene and wear protective footwear in tropical areas to avoid parasite exposure.
Avoid food allergens through diet and food allergy tests. Patient should participate in psychological counselling to manage their stress.
Proper awareness about HES should be provided and its related causes with management strategies.
Appointments with a physician and preventing recurrence of disorder is an ongoing life-long effort.
Hematology
Prednisone:
It prevents inflammation and controls rate of protein synthesis to suppress migration of polymorphonuclear leukocytes.
Hematology
Hydroxyurea:
It is used to reduce total white blood cells thus one week therapy may be required.
Etoposide:
It stabilizes the normal transient covalent intermediates between the DNA substrate and topoisomerase II.
Cytarabine:
It is converted intracellularly into the active compound cytarabine-5′-triphosphate to inhibit DNA polymerase.
Imatinib:
It inhibits the tyrosine kinase activity of bcr-abl kinase in Ph1-positive leukemic CML cell lines.
Hematology
Peginterferon alfa 2a:
It effectively suppresses eosinophilia in several patients using different doses.
Hematology
Mepolizumab:
It stops binding of IL-5 to its receptor on the surface of eosinophils.
Hematology
Valve replacement with bioprosthetic valves may be suggested in patients with HES.
Surgery necessary for patients with endomyocardial fibrosis or thrombosis.
Hematology
In the initial treatment phase, the goal is to manage eosinophil levels and stabilize the patient’s vital signs.
Pharmacologic therapy is effective in the treatment phase as it includes use of corticosteroids, antineoplastic agents, immunomodulators, and interleukin inhibitors.
In supportive care and management phase, patients should receive required attention such as lifestyle modification and surgical intervention.
The regular follow-up visits with the physician are scheduled to check the improvement of patients along with treatment response.
Hypereosinophilic Syndrome (HES) is a myeloproliferative disorder with persistent eosinophilia to cause organ damage.
Idiopathic HES diagnosis requires sustained AEC above 1500/µL for >6 months with tissue damage.
Hardy and Anderson described eosinophilia and tissue damage syndrome in 1968 after 80 years.
Genetic eosinophilia has stable eosinophil levels and mild symptoms. Overactive eosinophils cause tissue damage, inflammation, fibrosis, and organ malfunction.
HES is classified as:
Myeloproliferative
Lymphocytic
Idiopathic
Familial
Secondary eosinophilia is a cytokine-derived reactive phenomenon. Clonal eosinophilia is diagnosed using bone marrow histology and molecular genetics.
Idiopathic eosinophilia excludes secondary and clonal causes of elevated eosinophils. Autonomous proliferation causes chronic eosinophilic leukemia.
Idiopathic form is rarely noticed. In the US, allergies are the main cause of eosinophilia, while parasitosis is more common globally.
No racial predilection for HES syndrome while it has 9:1 male predominance ratio. Peak incidence shows in patients between 20 to 50 years old.
HES is rare in children and its incidence decreases in the elderly population.
Eosinophils survive in tissues for weeks with sustained cytokine presence. Only eosinophils, basophils, and precursors have specific receptors.
Eosinophils die within 48 hours without cytokines due to toxic cationic proteins in granules. Toxins include major basic protein and eosinophil-derived substances.
Eosinophilic peroxidase and respiratory burst lead to free radical production damaging tissues. HES syndrome causes organ damage from eosinophil infiltration and mediator release in tissues.
The causes of HES are:
Genetic Mutations
Cytokine Dysregulation
Immune System Dysfunction
Environmental and Infectious Triggers
It has an unpredictable outcome and can be deadly without treatment.
Idiopathic hypereosinophilic syndrome is generally mild, but patients with certain characteristics or heart failure fare worse.
Poor outcomes seen in HES with myeloproliferative features and leukocytosis over 90,000/ÎĽ L.
The syndrome has diverse complications based on affected organ systems during development.
Collect details including presenting symptoms, family and medical history to understand clinical history of patient.
Persistent urticaria, eczema, pruritus, gradual development of fatigue, peripheral edema, and dyspnea
Angiolymphoid hyperplasia
Atopic dermatitisc
Eosinophilic toxocariasis
Collagen vascular diseases
Not recommended to treat asymptomatic cases for hypereosinophilic syndrome due to high risks.
Patients are monitored with serum troponin every 3 to 6 months, for echocardiography and pulmonary tests every 6 to 12 months.
Initial treatment for patients without FIP1L1/PDGFRA mutation is glucocorticoids.
In unresponsive cases, use interferon alpha and hydroxyurea as second-line drugs.
Mepolizumab reduces flares in FIP1L1/PDGFRA-negative hypereosinophilic syndrome with interleukin-5 specificity.
Recurrent thromboembolic events happen in hypereosinophilic syndrome despite anticoagulant therapy.
Emergency leukapheresis in HES indicated to control symptoms from hyperleukocytosis.
Hematology
Use HEPA filters to reduce allergens and dehumidifiers to reduce mold growth.
Ensure proper ventilation in kitchens and bathrooms to prevent the humidity.
Follow good hand hygiene and wear protective footwear in tropical areas to avoid parasite exposure.
Avoid food allergens through diet and food allergy tests. Patient should participate in psychological counselling to manage their stress.
Proper awareness about HES should be provided and its related causes with management strategies.
Appointments with a physician and preventing recurrence of disorder is an ongoing life-long effort.
Hematology
Prednisone:
It prevents inflammation and controls rate of protein synthesis to suppress migration of polymorphonuclear leukocytes.
Hematology
Hydroxyurea:
It is used to reduce total white blood cells thus one week therapy may be required.
Etoposide:
It stabilizes the normal transient covalent intermediates between the DNA substrate and topoisomerase II.
Cytarabine:
It is converted intracellularly into the active compound cytarabine-5′-triphosphate to inhibit DNA polymerase.
Imatinib:
It inhibits the tyrosine kinase activity of bcr-abl kinase in Ph1-positive leukemic CML cell lines.
Hematology
Peginterferon alfa 2a:
It effectively suppresses eosinophilia in several patients using different doses.
Hematology
Mepolizumab:
It stops binding of IL-5 to its receptor on the surface of eosinophils.
Hematology
Valve replacement with bioprosthetic valves may be suggested in patients with HES.
Surgery necessary for patients with endomyocardial fibrosis or thrombosis.
Hematology
In the initial treatment phase, the goal is to manage eosinophil levels and stabilize the patient’s vital signs.
Pharmacologic therapy is effective in the treatment phase as it includes use of corticosteroids, antineoplastic agents, immunomodulators, and interleukin inhibitors.
In supportive care and management phase, patients should receive required attention such as lifestyle modification and surgical intervention.
The regular follow-up visits with the physician are scheduled to check the improvement of patients along with treatment response.
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