Case Report: We present the case of a patient who presented with confusion and was found to have an absolute eosinophil count of 8.08 × 109/L as well as clinical findings consistent with eosinophilic myocarditis. [ijcasereportsandimages.com]

At this stage patients often present with dyspnea, chest pain, signs of heart failure or new onset murmurs. Our case also had the unusual presentation of pericardial effusion. [indianpediatrics.net]

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

We report a rare case about a child with clinical presentation of HES whose bone marrow analysis indicated a diagnosis of leukemia. Male child, 7 years old, presenting abdominal pain and vomiting for four days. [oatext.com]

It manifests with varied clinical presentation and so, is essentially a diagnosis of exclusion. [ijdvl.com]

• Fever

  Due to the lymphadenopathies and fever, a chest X-ray was performed, which revealed pulmonary hyperinflation (Fig. 2). Figure 2 Chest X-ray with pulmonary hyperinflation. [scielo.org.mx]

  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]

  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]

  The clinical presentations include weakness, fatigue, cough, dyspnea, myalgia, angioedema, rashes, fever, rhinitis. [indianpediatrics.net]

  The M-HES can present with skin lesions in addition to fever, weight loss, fatigue, enlarged liver and spleen. Splinter hemorrhage may precede the thromboembolic disease when endomyocardial involvement is present. [dermatologyadvisor.com]

• Fatigue

  Early clinical features include fatigue, diarrhea, and rash. Prognosis is generally poor, and the 3-year survival rate of patients with hypereosinophilic syndrome is approximately 12%. [wikidoc.org]

  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]

  The M-HES can present with skin lesions in addition to fever, weight loss, fatigue, enlarged liver and spleen. Splinter hemorrhage may precede the thromboembolic disease when endomyocardial involvement is present. [dermatologyadvisor.com]

  This usually presents with fever, weight loss, fatigue, malaise, skin lesions and hepatosplenomegaly and responds to imatinib. Cardiac disease is more frequent, and the patient usually dies within 2 years of presentation. [ijdvl.com]

  The clinical presentations include weakness, fatigue, cough, dyspnea, myalgia, angioedema, rashes, fever, rhinitis. [indianpediatrics.net]

• Cough

  Case Report An 11-year-old boy was admitted with complaints of low-grade continuous fever, along with non productive, non spasmodic cough, bilateral diffuse chest pain increasing on coughing, generalized weakness and dyspnea for a period of 2 months. [indianpediatrics.net]

  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]

  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]

  Two months after, the patient was readmitted with a 24-h history of emetic cough and fever. No history of premedication or use of antibiotics or nonsteroidal anti-inflammatory drugs was registered. [scielo.org.mx]

• Dyspnea

  The clinical presentations include weakness, fatigue, cough, dyspnea, myalgia, angioedema, rashes, fever, rhinitis. [indianpediatrics.net]

  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]

  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]

  Acute eosinophilic pneumonia (AEP) Acute eosinophilic pneumonia (AEP) usually manifests with acute onset fever, severe dyspnea and hypoxia for <5 days with rapid progression and shows rapid improvement when treated with steroids and usually no relapse [radiopaedia.org]

• Diarrhea

  At the same time, the patient was being investigated for diarrhea and anemia; his bone marrow biopsy revealed marrow hyperplasia with increased eosinophilic precursors. [ijdvl.com]

  Early clinical features include fatigue, diarrhea, and rash. Prognosis is generally poor, and the 3-year survival rate of patients with hypereosinophilic syndrome is approximately 12%. [wikidoc.org]

  Hepatosplenomegaly, blurring vision and diarrhea are some of the less common presentations. Our patient had hepatosplenomegaly and pulmonary mani-festations. The primary aim of the management is to keep the eosinophilic counts low. [indianpediatrics.net]

  Gastrointestinal – abdominal pain, vomiting, and diarrhea. Musculoskeletal – arthritis, muscle inflammation, muscle aches, and joint pain. Nervous system – vertigo, paresthesia, speech impairment, and visual disturbances. [askhematologist.com]

  […] presented initially with incidentally detected asymptomatic hypereosinophilia Dermatologic: angioedema, dermographism, eczema, erythroderma, pruritis, urticaria Pulmonary: cough, dyspnea, wheezing Gastrointestinal: weight loss, abdominal pain, vomiting, diarrhea [pathologyoutlines.com]

• Abdominal Pain

  We report a case of a boy, 7 years old, presenting abdominal pain, vomiting, tachidispnea and leukocytosis with predominance of eosinophils. [oatext.com]

  If the digestive system is affected, the patient may suffer abdominal pain, diarrhoea or ascites (liquid in the abdominal cavity, which increases the abdominal perimeter). [marih.fr]

  pain and diarrhea with malabsorption have been described, explaining the underlying zinc deficiency in our case. [ijdvl.com]

  A travel history, particularly to areas where parasite infection is endemic, and gastrointestinal symptoms (including abdominal pain, diarrhoea, and nausea) raise the possibility of HE secondary to parasitisation. [emjreviews.com]

  Gastrointestinal – abdominal pain, vomiting, and diarrhea. Musculoskeletal – arthritis, muscle inflammation, muscle aches, and joint pain. Nervous system – vertigo, paresthesia, speech impairment, and visual disturbances. [askhematologist.com]

• Vomiting

  We report a case of a boy, 7 years old, presenting abdominal pain, vomiting, tachidispnea and leukocytosis with predominance of eosinophils. [oatext.com]

  Gastrointestinal – abdominal pain, vomiting, and diarrhea. Musculoskeletal – arthritis, muscle inflammation, muscle aches, and joint pain. Nervous system – vertigo, paresthesia, speech impairment, and visual disturbances. [askhematologist.com]

  The primary symptoms of EoE vary with age, with younger patients presenting with feeding difficulties, frequent vomiting, food refusal/selective eating, and failure to thrive (31). [frontiersin.org]

  […] patients presented initially with incidentally detected asymptomatic hypereosinophilia Dermatologic: angioedema, dermographism, eczema, erythroderma, pruritis, urticaria Pulmonary: cough, dyspnea, wheezing Gastrointestinal: weight loss, abdominal pain, vomiting [pathologyoutlines.com]

  Gastrointestinal Disorders Eosinophilic gastritis, enteritis, and/or colitis may occur secondary to HES and cause weight loss, abdominal pain, vomiting, and/or severe diarrhea [9]. Our patient had evidence of gastritis on esophagogastroduodenoscopy. [karger.com]

• Nausea

  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]

  A travel history, particularly to areas where parasite infection is endemic, and gastrointestinal symptoms (including abdominal pain, diarrhoea, and nausea) raise the possibility of HE secondary to parasitisation. [emjreviews.com]

  Case Presentation A 65-year-old African gentleman who was known to have asthma and who recently came from his home country presented with a 2-month history of central chest pain and epigastric pain associated with shortness of breath and nausea. [karger.com]

  Depending on the clinical presentation (e.g., nausea, vomiting, diarrhea, malabsorption, ascites), esophagogastroduodenoscopy (EGD) and colonoscopy with biopsies should be performed as the macroscopy is usually unremarkable. [link.springer.com]

  Symptoms depend on organ dysfunction and may include skin rashes, shortness of breath, chest pain/discomfort, abdominal pain/nausea/vomiting, or behavioral changes [2]. [ncbi.nlm.nih.gov]

• Ear Discharge

  There was no history of any rash, ear discharge, burning micturition, orthopnea, paroxysmal nocturnal dyspnea, wheezing, hematuria, anuria or dysuria. [indianpediatrics.net]

• Myalgia

  Offending drugs include: antibiotics nitrofurantoin (which may progress to pulmonary fibrosis) penicillin tetracycline toxins eosinophilia-myalgia syndrome from contaminated L-tryptophan toxic oil syndrome See also: drug rash with eosinophilia and systemic [radiopaedia.org]

  […] variant of HES), organ-restricted conditions accompanied by HE (eosinophilic gastrointestinal disorders, eosinophilic pneumonia, dermatologic diseases, etc.) and specific syndromes accompanied by HE (Gleich syndrome, Churg-Strauss syndrome, eosinophilia myalgia [journalpulmonology.org]

  The clinical presentations include weakness, fatigue, cough, dyspnea, myalgia, angioedema, rashes, fever, rhinitis. [indianpediatrics.net]

  Clinically, patients with SHS may present dyspnea, weakness, cough, myalgias, fever and cutaneous rash. The common laboratory finding is leukocytosis (above 20,000/mm3) with eosinophils predominance [3]. [oatext.com]

  Toxins: eosinophilia-myalgia syndrome from ingestion of contaminated L-tryptophan, toxic oil syndrome from ingestion of adulterated oil with denatured rapeseed oil. Sarcoidosis. Inflammatory bowel disease. [cancertherapyadvisor.com]

• Angioedema

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

  The clinical presentations include weakness, fatigue, cough, dyspnea, myalgia, angioedema, rashes, fever, rhinitis. [indianpediatrics.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]

  These patients often show predominant cutaneous manifestations including pruritus, eczema, erythroderma, urticaria, and angioedema. [jsstd.org]

• Urticaria

  Dermatologists should be alert to pruritic erythematous papules, urticaria, angioedema, dermographism, oral and genital ulcers, centrifugal annular erythema, acral bullae, and erythroderma. [anaisdedermatologia.org.br]

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

  These patients often show predominant cutaneous manifestations including pruritus, eczema, erythroderma, urticaria, and angioedema. [jsstd.org]

  Lymphocytic form presents with pruritus, eczema, erythroderma, urticaria, angioedema and lymphadenopathy. Lymphocytic hypereosinophilic syndrome follows a more benign course.[3] There is often a raised serum immunoglobulin E level. [ijdvl.com]

  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]

• Eczema

  […] systemic mastocytosis) à next test is KIT on bone marrow BMAT and cytogenetics Assess end organ damage CT, Echo, Troponin, Lung function tests Reactive / Secondary Eosinophilia Causes of Secondary / Reactive Eosinophilia Allergic – Asthma, dermatitis, eczema [haembase.com]

  Lymphocytic form presents with pruritus, eczema, erythroderma, urticaria, angioedema and lymphadenopathy. Lymphocytic hypereosinophilic syndrome follows a more benign course.[3] There is often a raised serum immunoglobulin E level. [ijdvl.com]

  These patients often show predominant cutaneous manifestations including pruritus, eczema, erythroderma, urticaria, and angioedema. [jsstd.org]

  Physical examination may be remarkable for:[3] Skin rash Thickening of the skin (lichenification) Eczema (flexural areas) Dermographism Low-grade fever Raynaud phenomenon Wheezing Laboratory Findings[edit] Laboratory findings consistent with the diagnosis [wikidoc.org]

  The nature of skin symptoms is highly variable, with some having cutaneous erythema or eczema while others may have more severe symptoms such as urticaria, angioedema, or even ulceration. [emjreviews.com]

• Pruritus

  Six years previously he presented intermittent erythematous papules on the trunk and face, in addition to pruritus aggravated by heat or alcohol intake. [anaisdedermatologia.org.br]

  The patient also complained about dysphagia, intense pruritus and occasional dyspnea, the latter was alleviated with salbutamol and the pruritus was relieved with dexchlorpheniramine. The patient was readmitted due to intense dysphagia. [htct.com.br]

  Marked to intolerable permanent pruritus is a common indicative symptom of hypereosinophilia syndromes. Thus, the dermatologist is involved early in the diagnostic process. [altmeyers.org]

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

  These patients often show predominant cutaneous manifestations including pruritus, eczema, erythroderma, urticaria, and angioedema. [jsstd.org]

• Papule

  Six years previously he presented intermittent erythematous papules on the trunk and face, in addition to pruritus aggravated by heat or alcohol intake. [anaisdedermatologia.org.br]

  Case 1: A 55-year-old female patient presented with pruritic papules, vesicles, and tense bullae of 1-year duration. [jsstd.org]

  Cutaneous manifestations include urticarial lesions, angioedema, purpuric papules and nodules, cutaneous micro thrombi. [indianpediatrics.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]

  Angiolymphoid hyperplasia with eosinophilia: Presents with eosinophilia and papules, plaques, and nodules on the head and neck. Kimura disease presents with eosinophilia and large subcutaneous masses on the head or neck. Typically seen in Asian men. [unboundmedicine.com]

• Dysuria

  There was no history of any rash, ear discharge, burning micturition, orthopnea, paroxysmal nocturnal dyspnea, wheezing, hematuria, anuria or dysuria. [indianpediatrics.net]

• Burning Micturition

  There was no history of any rash, ear discharge, burning micturition, orthopnea, paroxysmal nocturnal dyspnea, wheezing, hematuria, anuria or dysuria. [indianpediatrics.net]

• Stroke

  She underwent repeat head CT imaging, which revealed acute ischemic multi-territory infarcts consistent with embolic strokes. The heparin drip was discontinued to prevent hemorrhagic conversion of the ischemic strokes. [ijcasereportsandimages.com]

  Nallegowda M, Sing U, Handa G, B Arora (2003) Idiopathic Hypereosinophilic Syndrome with Stroke in Young: A Case Report. IJPMR 14: 24-26. [clinmedjournals.org]

  Neurologic manifestations range from peripheral neuropathy to stroke or encephalopathy. Bone marrow examination reveals increased eosinophil precursors. [unboundmedicine.com]

  Cerebral thromboemboli can arise from intracardiac thrombi and manifest as embolic strokes or transient ischemic episodes. MRI can reveal multiple infarcts in watershed areas [8], as seen in our case. [karger.com]

  In addition, there are some case reports of patients with HES presenting with embolic stroke [7]. [cureus.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]

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]

A full workup is mandatory before making a diagnosis of HES. Failure to detect the condition early may lead to irreversible cardiac damage. In view of its potential to involve other systems, a multidisciplinary approach is essential in managing HES. [jsstd.org]

As an additional workup, no lymphocyte clonality was found by flow cytometry. [htct.com.br]

[…] splenomegaly and lymphadenopathy Liver, CNS, muscle, pulmonary and nasal sinus involvement occur more commonly than in eosinophilic leukemia (Haematologica 2014;99:e148) Etiology Cause is unknown Diagrams / tables Images hosted on other servers: Algorithm workup [pathologyoutlines.com]

• Ischemic Changes

  Her troponin peaked at 5.70 ng/mL on day 3 of hospitalization and the EKG was without ischemic changes. [ijcasereportsandimages.com]

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

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

Emergency treatment 7. Treatment of primary (clonal) eosinophilic disorders 8. Treatment of lymphocyte variant HES 9. Treatment of idiopathic HES Declaration of Interests The BSH paid the expenses incurred during the writing of this guidance. [b-s-h.org.uk]

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

Prognosis varies depending on the response to therapy. Response to imatinib improves the prognosis among patients with the FIP1L1/PDGFRA-associated fusion gene and other responsive gene fusions. Current therapy has improved the prognosis. [askhematologist.com]

Genotyping excludes myeloproliferative disease, thereby orienting treatment and prognosis. [anaisdedermatologia.org.br]

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]

Acquired blood eosinophilia (much more prevalent than familial eosinophilia) may be of primary etiology or secondary to a wide variety of disorders. [cancertherapyadvisor.com]

Most cases of pediatric HE have an underlying secondary etiology. Infection Normal immunological responses to certain infectious pathogens remain among the most common etiologies of secondary HE in children worldwide. [frontiersin.org]

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

The etiology of AEP is unknown. It has been suggested that it may represent acute hypersensitivity to an inhaled antigen. CT findings include bilateral patchy areas of ground-glass opacity and interlobular septal thickening. [radiopaedia.org]

Eosinophilia of any etiology can cause the same types of tissue damage. Hypereosinophilic syndrome is rare, has an unknown prevalence, and most often affects people aged 20 through 50. [askhematologist.com]

Biology and epidemiology of eosinophilia 2. Secondary (reactive) eosinophilia and its causes 3. Clonal eosinophilia 4. Idiopathic hypereosinophilia and the idiopathic hypereosinophilic syndrome (HES) 5. [b-s-h.org.uk]

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]

Epidemiology The overall prevalence of HE within the pediatric population remains unknown as no population-based studies have been completed. [frontiersin.org]

[…] age-adjusted incidence rate of hypereosinophilic syndrome–a type of eosinophilic disorder–was approximately 0.4 cases per 1,000,000 according to the International Classification of Disease for Oncology (version 3), and coding of 9964/3, the Surveillance, Epidemiology [consultant360.com]

Epidemiology In industrialized nations, peripheral blood eosinophilia is most often due to atopic disease, whereas helminthic infections are the most common cause of eosinophilia in the rest of the world. [unboundmedicine.com]

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

Eosinophil granule proteins, including eMBP1, have various and numerous toxic effects on tissues and organs, and their detection has important pathophysiologic and treatment implications. [arupconsult.com]

The pathophysiology and clinical presentation of eosinophilic disorders are highly heterogeneous and disease outcome may vary from asymptomatic or mild, to severe and fatal, with variable time course patterns. [ctajournal.biomedcentral.com]

The role of eosinophils as secretory cells and mediators of the cytotoxic response seems to explain the pathophysiology of peripheral neuropathy, particularly major basic protein and eosinophil-derived neurotoxin, which are responsible for neuropathic [elsevier.es]

Their development depends entirely on which organ systems are involved in the disease process (see Presentation and Pathophysiology). [emedicine.medscape.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]

The patient was deemed too unstable to undergo cardiac magnetic resonance imaging (MRI) and anticoagulation for prevention of cardiac thrombus formation was deferred due to her recent stroke. [ijcasereportsandimages.com]

Treatment Treatment goals include decreasing blood eosinophil numbers, preventing organ damage, and slowing disease progression. [apfed.org]

Antibodies targeting IL-5 Monoclonal anti-IL-5 antibodies target eosinophils by binding to IL-5 and preventing its ligation to the IL-5R α-chain expressed on the eosinophil membrane. [tandfonline.com]