Conclusion In conclusion, HES still presents difficulties in both diagnosis and management, while it may present with potentially life-threatening complications. [sciencerepository.org]

No ring sideroblasts are present. Scattered mature mast cells are present (2%). [pathologyoutlines.com]

Case Report A 46-year-old white woman presented with double vision, mild ptosis, and markedly decreased vision in her left eye. [ajnr.org]

A follow-up examination at 6 months revealed no motor or sensory impairment, with the patient presenting a normal blood eosinophil count. [elsevier.es]

"Hypereosinophilic Syndrome Presenting As Pruriginous Erythroderma". [dergipark.org.tr]

• Splenomegaly

  Organ involvement (e.g., splenomegaly) is frequently present, sometimes associated with organ dysfunction, especially of the heart. [link.springer.com]

  Other common complications include central or peripheral nervous system involvement, hepato- and/or splenomegaly, and coagulation disorders. [orpha.net]

  FDA-regulated Device Product: No Product Manufactured in and Exported from the U.S.: No Additional relevant MeSH terms: Layout table for MeSH terms Hepatomegaly Hypereosinophilic Syndrome Eosinophilia Syndrome Splenomegaly Disease Pathologic Processes [clinicaltrials.gov]

  Ultrasound abdomen helps to evaluate for splenomegaly. Stool testing helps to assess for parasitic infections. Treatment / Management Management depends on the underlying cause. [statpearls.com]

  People with PDGFRA-associated chronic eosinophilic leukemia can also have an enlarged spleen (splenomegaly) and elevated levels of certain chemicals called vitamin B12 and tryptase in the blood. [medlineplus.gov]

• Anemia

  Morphologically, RBCs show normochromic, normocytic anemia with mild anisopoikilocytosis. WBCs: slightly increased in number (13000/µL) without neutrophilia, blastosis or immature monocytosis. [pathologyoutlines.com]

  Symptoms include skin rashes, itching, asthma, difficulty breathing, abdominal pain, vomiting, diarrhea, arthritis, muscle inflammation, congestive heart failure, deep venous thrombosis (blood clots in the veins) and anemia. [fda.gov]

  Repeat testing may be indicated for eosinophilia (typically considered >500 cells/µL) but may not be required if absolute counts exceed 1,500 cells/µL, especially in a symptomatic patient (anemia and thrombocytopenia detected on CBC may indicate a neoplasm [arupconsult.com]

  Findings related with poor prognosis in hypereosinophilic syndrome, include: presence of anemia, thrombocytopenia, white blood cell count greater than 100,000 cells/cm3, abnormal marrow and/or basophils, elevated serum levels of vitamin B12, serum tryptase [wikidoc.org]

  Eosinophilic leukemia is a rare disease characterized by progressive anemia, lymphadenopathy, hepatosplenomegaly, and increased eosinophils that show abnormal granulation in peripheral blood and bone marrow. [contemporarypediatrics.com]

• Eosinophilic Asthma

  Relationship of inhaled corticosteroid adherence to asthma Eeacerbations in patients with moderate-to-severe asthma. J Allergy Clin Immunol Pract. 2018;6(6):1989-1998.e3. doi:10.1016/j.jaip.2018.03.008 8. [consultant360.com]

  In conditions where eosinophilia is considered to play an important part in the pathology, including eosinophilic asthma, HES, and eosinophilic granulomatosis with polyangiitis, a consistent reduction in blood eosinophil counts is observed in association [mayo.edu]

  […] granuloma, a benign tumor on the bone allergies rhinitis and other upper respiratory diseases asthma and eosinophilic asthma other lower respiratory diseases immune-mediated diseases, such as systemic sclerosis and primary biliary cirrhosis atopic dermatitis [medicalnewstoday.com]

  FDA approved the drug for use as add-on maintenance therapy for patients with severe eosinophilic asthma. [nejm.org]

  Hypereosinophilia with asthma and systemic (non-vasculitic) manifestations: eosinophilic granulomatosis with polyangiitis or hypereosinophilic syndrome ? Autoimmun Rev. 2017;16(2):208–9. https://doi.org/10.1016/j.autrev.2016.11.001. [ctajournal.biomedcentral.com]

• Constitutional Symptom

  Constitutional symptoms of fever, myalgia and fatigue may occur. Other common complications include central or peripheral nervous system involvement, hepato- and/or splenomegaly, and coagulation disorders. [orpha.net]

  Constitutional symptoms like low-grade fevers, night sweats, fatigue, weight loss can occur in multiple conditions, including myeloproliferative and lymphoid neoplasms, Churg Strauss syndrome, DRESS syndrome. [statpearls.com]

  Case Report A 41-year-old woman presented to the emergency department with constitutional symptoms and diffuse arthralgias waxing and waning for a few weeks. [sciencerepository.org]

  The presence of constitutional symptoms (night sweats, fever, >10% unintended weight loss) should prompt consideration of underlying malignancy. [emjreviews.com]

  Systemic or constitutional symptoms are common and include fatigue, fever, weight loss/gain and pruritus among others. Skin, heart, lung, and nervous system are involved in majority of patients. The eosinophilia may also be an incidental finding. [dermatologyadvisor.com]

• Cough

  He also had respiratory symptom of chronic cough. He was taking no medications, including herbs and drugs, and had no history of food or drug allergies. [e-ce.org]

  Common and early HES symptoms include fatigue, cough, shortness of breath, myalgia, skin rash, and fever. Symptoms of hypereosinophilia correlate with areas of the body affected by high levels of eosinophils. [icliniq.com]

  You have chills, a cough, or feel weak and achy. You have questions or concerns about your condition or care. When should I seek immediate care or call 911? You have severe chest or abdominal pain. You have sudden trouble breathing. [drugs.com]

  Symptoms Early symptoms of HES may include fatigue, cough, breathlessness, muscle pain, rash and fever. Causes Some varieties of hypereosinophilic syndrome tend to run in families. [mayoclinic.org]

• Dyspnea

  During the hospital stay, the dyspnea worsening and a new thorax CT was performed, which revealed a 7.0cm diameter cavity in the right hemithorax. [htct.com.br]

  Any organ or tissue may be affected and myalgia, arthritis, dyspnea, pruritus, skin lesions and other symptoms may develop.33 Gotlib J. [scielo.br]

  Acute eosinophilic pneumonia is an idiopathic disease that presents with fever, cough, dyspnea, and hypoxemia occurring for days to weeks, typically in males and in individuals who have recently started to smoke tobacco. [unboundmedicine.com]

  Common manifestations include brain or lung hypoxia (eg, encephalopathy, dyspnea or respiratory failure). 1. [msdmanuals.com]

  • Cardiac involvement: The patient reported chest pain and dyspnea. Blood analyses demonstrated elevation in high-sensitive troponin (0.5 ng/mL), as well as NT-pro-BNP (10.000 pg/mL). [sciencerepository.org]

• Early Satiety

  The clinical manifestations of EGIDs vary depending on the site of the affected GI tract and the extent of involvement and include abdominal pain, nausea, vomiting, early satiety, diarrhea, weight loss with protein-losing enteropathy, rectal bleeding, [lidsen.com]

• Thrombosis

  Since in HES patients about half (45%) of thrombosis were arterial,[29] combination of anticoagulants with an antiplatelet agent may still have a role in preventing the recurrence of thrombosis in HES. [journals.lww.com]

  Thrombotic complications in clonal eosinophilic disorders have been described in case reports but the prevalence of thrombosis has not been extensively studied. [ash.confex.com]

  The majority of people with HES do not require treatment but require careful monitoring for organ damage, development of blood clots (thrombosis), and disease progression. If there is organ involvement, steroids are first line therapy. [verywellhealth.com]

  Colour ultrasound showed ascites, hepatosplenomegaly, superficial lymph node enlargement, and superficial venous thrombosis. [tandfonline.com]

• Cardiomegaly

  Cardiac Disease Later thrombotic & fibrotic stages  Signs & Symptoms - Dyspnea - Chest pain - Signs of LV&RV CHF - Murmurs of atrioventricular valve regurgitation - Cardiomegaly Klion AD. et al.Middleton's Allergy ; 8th edition. 2014. p. 1205-23. 79. [slideshare.net]

• Hepatosplenomegaly

  Some possible symptoms a patient may present with include: Cardiomyopathy[5] Skin lesions[5] Thromboembolic disease[5] Pulmonary disease[5] Neuropathy[5] Hepatosplenomegaly[5] Reduced ventricular size[5] Atopic eczema Diagnosis[edit] Numerous techniques [en.wikipedia.org]

• Hepatomegaly

  White Cells Blasts More Than 5 Percent of Bone Marrow Nucleated Cells Blasts More Than 5 Percent of Peripheral Blood White Cells Blasts Under 20 Percent of Bone Marrow Nucleated Cells Chronic Eosinophilic Leukemia, Not Otherwise Specified Eosinophilia Hepatomegaly [clinicaltrials.gov]

  He had no kidney injury, hepatomegaly or splenomegaly. Ceftriaxone and azithromycin were prescribed to treat communitary pneumonia and the prednisone dosage was reduced to 15mg/day. [htct.com.br]

  He had no kidney injury, hepatomegaly or splenomegaly. Figure 1 A and B before Imatinib. Ceftriaxone and azithromycin were prescribed to treat communitary pneumonia and the prednisone dosage was reduced to 15 mg/day. [scielo.br]

  Classification Myeloproliferative forms of HES • Clinical Hepatomegaly, Splenomegaly • Laboratory - Circulating myeloid precursors, Increased serum vitamin B12 or Tryptase, Anemia, Thrombocytopenia - Hematologic (myeloid fibrosis, left shift in maturation [slideshare.net]

  Myeloid Hypereosinophilic Syndrome Patients with M-HES are characterized by hepatomegaly, splenomegaly, circulating myeloid precursors, increased serum vitamin B12 and/or tryptase levels, anemia, thrombocytopenia, hematologic diseases (myeloid fibrosis [mdpi.com]

• Ulcer

  Mucosal ulcerations have been reported as a distinct feature of M-HES. [dermatologyadvisor.com]

  This time there were pustules and ulcers of the oral mucosa, and on the elbows and back (Figure 3). [htct.com.br]

  Leiferman KM O'Duffy JD Perry HO Greipp PR Giuliani ER Gleich GJ Recurrent incapacitating mucosal ulcerations: a prodrome of the hypereosinophilic syndrome. [thelancet.com]

  Dermatological features are common and very variable and include pruritus, skin rash, nodules, mucosal ulcerations and oedema. [therapeutique-dermatologique.org]

  Skin manifestations vary greatly: pruritus, skin rashes, nodules, mucous ulcerations, and oedema. [marih.fr]

• Angioedema

  Cytokine-associated angioedema syndromes including episodic angioedema with eosinophilia (Gleich's Syndrome). Immunology and allergy clinics of North America. 2006;26(4):769-81. 66. [slideshare.net]

  Cyclic angioedema with eosinophilia falls in the latter category. [dermatologyadvisor.com]

  Clinical description Target-organ damage mediated by eosinophils is highly variable among patients, and consists of dermatological involvement (urticaria, eczema, angioedema, pruriginous papules, nodules, erythroderma) in more than 50% of cases, followed [orpha.net]

  Pruritus, urticarial erythema, papulovesicles, red papules and nodules, angioedema (see also Angioedema, episodic with eosinophilia), petechiae and lichenification are seen. In rare cases, erythroderma may develop. [altmeyers.org]

  Episodic angioedema with eosinophilia (Gleich syndrome) is a rare disease that leads to recurrent attacks of fever, angioedema, urticaria, weight gain, and blood eosinophilia without other organ damage. [unboundmedicine.com]

• Urticaria

  It can also resemble urticaria (hives). An enlarged liver and spleen is often present indicating liver and spleen involvement. The lungs, kidneys, heart and nervous system can be affected. [dermnetnz.org]

  Clinical description Target-organ damage mediated by eosinophils is highly variable among patients, and consists of dermatological involvement (urticaria, eczema, angioedema, pruriginous papules, nodules, erythroderma) in more than 50% of cases, followed [orpha.net]

  Eczema, erythroderma, urticaria and angioedema along with lymphadenopathy are common in this type. [dermatologyadvisor.com]

  Skin Manifestation • Angioedema & urticaria • Pruritic papules or nodules • Cutaneous microthrombi or digital arteritis • Mucosal ulcers & lymphomatoid papulosis Klion AD. et al.Middleton's Allergy ; 8th edition. 2014. p. 1205-23. 89. [slideshare.net]

  Urticaria, eczema, and atopic dermatitis are the most common dermatologic conditions associated with eosinophilia. Typical historical and physical features are usually enough for making these clinical diagnoses. [contemporarypediatrics.com]

• Sweating

  Symptoms Symptoms of eosinophilia are generally related to the underlying condition, and may include: • Wheezing • Breathlessness • Coughing • Abdominal pain • Diarrhea • Fever • Rashes • Weight loss • Night sweats • Enlarged lymph nodes • Numbness and [cancer.columbia.edu]

  […] test MRI Scan Symptoms The symptoms associated with hypereosinophilic syndrome are listed below: Skin itching and redness Abdominal pain Joint and muscle pain Fever Chest pain Fatigue Rhinitis Shortness of breath Cough Blurry vision Dizziness Night sweats [anavara.com]

  If HES affects your heart, you should go to the emergency room any time you have symptoms such as chest pain, heartburn, nausea and sweating. What questions should I ask my doctor? Hypereosinophilic syndrome is a rare medical condition. [my.clevelandclinic.org]

  Recently, he has had night sweats heavy enough to completely saturate his bedding. Since a physical examination before camp six months ago, he has lost seven pounds. [contemporarypediatrics.com]

  Inflammation of the mucous membranes of the nose (rhinitis), unintended weight loss and night sweats have also been reported. Some individuals with SPE develop acute eosinophilic pneumonia (AEP). [rarediseases.org]

• Night Sweats

  Symptoms Symptoms of eosinophilia are generally related to the underlying condition, and may include: • Wheezing • Breathlessness • Coughing • Abdominal pain • Diarrhea • Fever • Rashes • Weight loss • Night sweats • Enlarged lymph nodes • Numbness and [cancer.columbia.edu]

  sweats Irregular bowel movements Nausea Symptoms depend upon the age and which part of the body is connected with this syndrome. [anavara.com]

  Recently, he has had night sweats heavy enough to completely saturate his bedding. Since a physical examination before camp six months ago, he has lost seven pounds. [contemporarypediatrics.com]

  Inflammation of the mucous membranes of the nose (rhinitis), unintended weight loss and night sweats have also been reported. Some individuals with SPE develop acute eosinophilic pneumonia (AEP). [rarediseases.org]

  Constitutional symptoms like low-grade fevers, night sweats, fatigue, weight loss can occur in multiple conditions, including myeloproliferative and lymphoid neoplasms, Churg Strauss syndrome, DRESS syndrome. [statpearls.com]

Testing for Secondary/Reactive Eosinophilia A broad range of conditions can result in secondary/reactive eosinophilia, and a clinically driven workup is needed to determine cause. [arupconsult.com]

Workup for clonal eosinophilia including research for PDGFR-α, BCR-ABL, c-kit and JAK-2 rearrangements was negative. [sciencerepository.org]

[…] in tissue (defined by 1 or more of the following): Bone marrow aspiration with >20% eosinophils Histologically proven tissue infiltration Deposition of eosinophil-granule proteins Sustained hypereosinophilia without an apparent cause should prompt a workup [eosinophilosophy.com]

Histological workup showed infiltration by eosinophilic granulocytes and no evidence of malignancy. Bronchoscopy with BAL and transbronchial biopsy showed evidence of eosinophils without granuloma formation. [link.springer.com]

Specifically, lung involvement is evaluated using pulmonary function testing, bronchoscopy, and serologic tests.1 Once any secondary causes are excluded, the workup should evaluate for primary (clonal) eosinophilia. [consultant360.com]

• Thrombocytosis

  Laboratory findings that may indicate clonal eosinophilia include additional blood count changes, such as left-shifted leukocytosis, blasts, monocytosis, or thrombocytosis, and elevations of vitamin B12 (nonspecific but frequently elevated in myeloid [link.springer.com]

  Comparison of the Mutational Profiles of Primary Myelofibrosis, Polycythemia Vera, and Essential Thrombocytosis. Am. J. Clin. Pathol. 2017, 147, 444–452. [mdpi.com]

• Neutrophilia

  WBCs: slightly increased in number (13000/µL) without neutrophilia, blastosis or immature monocytosis. Occasional circulating immature granulocytic precursors, e.g. metamyelocytes and myelocytes, are noted. [pathologyoutlines.com]

• Erythroblast

  BCR = breakpoint cluster region; CC = Coiled Coil; GEF = Guanine Nucleotide Exchange Factor; ETS = (Erythroblast Transformation Specific) DNA-binding domain; HLH = Helix-Loop-Helix oligomerization domain; FERM = 4.1 ezrin, radixin and moesin domain; JAK2 [mdpi.com]

• Toxocara Canis

  Among the helminths, the principal parasites that need to be evaluated are S. stercoralis, hookworm, and Toxocara canis. The diagnostic consideration can also vary according to geographic region. [unboundmedicine.com]

  If eosinophilia is present without an obvious cause, the following tests should be done: stool examination for ova and parasites serologic evaluation for parasites not detectable by stool examination, such as Toxocara canis atopy and tuberculin testing [contemporarypediatrics.com]

  In vivo changes of hemopoietic progenitors and the expression of the interleukin 5 gene in eosinophilic mice infected with Toxocara canis. Exp Hematol. 1990;18:1152-1157. 6. Johnson BE. Paraneoplastic syndromes. [hematologyandoncology.net]

• Hypercellular Bone Marrow

  Another myelogram showed hypercellular bone marrow with 78% of cells being granulocytes (27% eosinophils and 51% eosinophil precursors) of hypoblastic erythroid lineage and hypercellular megakaryocyte lineage, presenting monolobulation and polylobulation [htct.com.br]

Monitoring the response of skin lesions to treatment helps evaluate the systemic efficacy of the treatment. [dermatologyadvisor.com]

Experimental: Treatment (ruxolitinib) Patients receive ruxolitinib PO BID on days 1-28. [clinicaltrials.gov]

(A,D) before treatment, (B,E) after 7 days of treatment, (C,F) after 7 weeks of treatment (patients from the parasite HES group). [tandfonline.com]

Future treatments may involve the JAK1/2 inhibitor ruxolitinib (and other JAK inhibitors). [consultant360.com]

Cardiovascular complications of HES represent the worst prognosis, responsible for a high rate of fatal outcome. [ctajournal.biomedcentral.com]

Prognosis Prognosis has improved significantly since defining HES, and currently depends on the development of irreversible endomyocardial fibrosis, as well as eventual malignant transformation of myeloid or lymphoid cells. [orpha.net]

Prognosis is generally poor, and the 3 year survival rate of patients with hypereosinophilic syndrome is approximately 12% Findings related with poor prognosis in hypereosinophilic syndrome, include: presence of anemia, thrombocytopenia, white blood cell [wikidoc.org]

Prognosis Hypereosinophilic syndrome carries a variable prognosis. It is a chronic and progressive disorder that is potentially fatal if left untreated. Blast transformation may occur after many years. [emedicine.medscape.com]

What is the prognosis of hypereosinophilic syndrome? The prognosis of this syndrome depends upon variable factors. In severe or chronic cases, it can be fatal if left untreated. [anavara.com]

Based on the etiology, the outcome can be fatal and cause severe end-organ damage. [statpearls.com]

This syndrome of unknown etiology occurs 90% in men and 10% in females.[1],[2],[3] We report one case of hypereosinophilic syndrome (HES) in a female. [ijdvl.com]

Clinically there are no identifiable etiologies of reactive eosinophilia such as allergic reactions, parasitic infections, autoimmune diseases and medications. [pathologyoutlines.com]

Hypereosinophilic syndrome (HES) is a rare group of disorders characterized by peripheral hypereosinophilia with eosinophil-mediated organ dysfunction and/or damage, with no other identifiable etiology for eosinophil-mediated organ damage.1,2 US estimated [eosinophilosophy.com]

Consistent with an allergic etiology, SGCs are effective for the treatment of EoE and elicit local changes in esophageal expression. [www1.rarediseasesnetwork.org]

Summary Epidemiology Prevalence is unknown. It frequently occurs in middle-aged patients, but may concern any age group. [orpha.net]

Causes of reactive eosinophilia have a significant regional influence worldwide and regional large epidemiologic studies would be needed for a global evaluation of the problem. [ctajournal.biomedcentral.com]

[…] may be treated with the monoclonal antibody mepolizumab currently being developed to treat the disease.[7] If this becomes successful, it may be possible for corticosteroids to be eradicated and thus reduce the amount of side effects encountered.[7] Epidemiology [en.wikipedia.org]

Hypereosinophilic syndromes: Clinical manifestations, pathophysiology and diagnosis. https://www.uptodate.com/contents/search. Accessed April 22, 2020. Roufosse F, et al. [mayoclinic.org]

There is still much uncertainty in the diagnosis and management of HES, and research should be done to improve knowledge on pathophysiology and cytogenetics of this disease in order to improve treatment and prevent life-threatening complications. [sciencerepository.org]

Areas of uncertainty: Little is known regarding the natural history and pathophysiological mechanisms, especially for EG and EC, and FDA-approved drugs do not exist for any of these diseases. [www1.rarediseasesnetwork.org]

Hypereosinophilic syndromes: Clinical manifestations, pathophysiology, and diagnosis and Hypereosinophilic syndromes: Treatment. In: UpToDate, Post TW (Ed), UpToDate, Waltham, MA. [verywellhealth.com]

Clinical trials Explore Mayo Clinic studies testing new treatments, interventions and tests as a means to prevent, detect, treat or manage this condition. [mayoclinic.org]

FAQ Can hypereosinophilic syndrome be prevented? No, hypereosinophilic syndrome cannot be prevented. What is the prognosis of hypereosinophilic syndrome? The prognosis of this syndrome depends upon variable factors. [anavara.com]

If you provide content to customers through CloudFront, you can find steps to troubleshoot and help prevent this error by reviewing the CloudFront documentation. [astrazeneca.com]

Prevention[edit] There are no primary preventive measures available for hypereosinophilic syndrome.[6] There are no effective measures for the primary prevention of hypereosinophilic syndrome. [wikidoc.org]