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]

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

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]

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]

• 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]

  For example, eosinophils play a key role in the symptoms of asthma and allergies, such as hay fever. Other immune system disorders also can contribute to ongoing (chronic) inflammation. [mayoclinic.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]

  Other clinical features include fever, hepatosplenomegaly, gastroenteritis, and coagulation disorder [1,2]. [jrd.or.kr]

• Fatigue

  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]

  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]

  Hypereosinophilic syndrome usually presents with fever, weight loss, fatigue, and rash. A rash is present in over 50% of patients, but is non-specific in appearance. Most commonly, red swollen itchy nodules (lumps) have been described. [dermnetnz.org]

  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]

  Syndrome [LHES] (effective October 01, 2020) D72.118 Other Hypereosonophilic syndrome (effective October 01, 2020) D72.119 Hypereosonophilic syndrome (effective October 01, 2020) Signs and Symptoms Angioedema (rapid swelling of the dermis) Cough Diarrhea Fatigue [seer.cancer.gov]

• Chronic Infection

  The disorder can derive from non-neoplastic conditions (such as chronic infections and infestations, allergic reactions, intoxications, or autoimmune and chronic inflammatory disorders) or from neoplasms including non-myeloid malignancies, among others [orpha.net]

  In cases of blood eosinophilia, Strongyloides stercoralis infection must be excluded because this helminth can set up a cycle of autoinfection leading to chronic infection with intermittent, sometimes marked, eosinophilia. [unboundmedicine.com]

  The differential diagnosis also includes celiac disease, chronic granulomatous disease, connective tissue disorders and vasculitides, multiple infections (particularly parasites), hypereosinophilic syndrome, early inflammatory bowel disease, and rarely [slideshare.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]

  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]

  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]

  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]

• 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]

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

  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]

• 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]

  Mucosal involvement can produce nausea, vomiting, diarrhea, abdominal pain, gastrointestinal bleeding, protein-losing enteropathy, or malabsorption. [slideshare.net]

  CASE REPORT A 56-year-old man visited the hospital with complaints of more than 30 bloody diarrheas per day, combined with lower abdominal pain and febrile sensation for 5 days. [e-ce.org]

  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]

  […] dermatological involvement (urticaria, eczema, angioedema, pruriginous papules, nodules, erythroderma) in more than 50% of cases, followed by involvement of lungs (cough, breathlessness and wheezing) and digestive tract (nausea, vomiting, abdominal pain, diarrhea [orpha.net]

• Abdominal Pain

  Mucosal involvement can produce nausea, vomiting, diarrhea, abdominal pain, gastrointestinal bleeding, protein-losing enteropathy, or malabsorption. [slideshare.net]

  Generalised symptoms - fatigue, aches and pains, fever, night sweats and pruritus. Diarrhoea is common, as are abdominal pain and nausea. [patient.info]

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

  pain, diarrhea, ascites) in roughly 40%. [orpha.net]

  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]

• Vomiting

  Mucosal involvement can produce nausea, vomiting, diarrhea, abdominal pain, gastrointestinal bleeding, protein-losing enteropathy, or malabsorption. [slideshare.net]

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

  […] patients, and consists of dermatological involvement (urticaria, eczema, angioedema, pruriginous papules, nodules, erythroderma) in more than 50% of cases, followed by involvement of lungs (cough, breathlessness and wheezing) and digestive tract (nausea, vomiting [orpha.net]

  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]

  Skin: eczema, thickening of the skin, hives, Lungs: cough, shortness of breath, wheezing Heart: chest pain, difficulty breathing, Neurologic disease Blood clots (thrombosis) Eyes: Blurred vision Gastrointestinal (GI) tract: weight loss, abdominal pain, vomiting [verywellhealth.com]

• Nausea

  Alcohol intolerance with abdominal pain, flushing, and nausea. Organ involvement in HES[6] This can be manifold and the following are some examples: Blood - thrombocytopenia, hypercoagulability. [patient.info]

  […] among patients, and consists of dermatological involvement (urticaria, eczema, angioedema, pruriginous papules, nodules, erythroderma) in more than 50% of cases, followed by involvement of lungs (cough, breathlessness and wheezing) and digestive tract (nausea [orpha.net]

  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]

  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]

  Mucosal involvement can produce nausea, vomiting, diarrhea, abdominal pain, gastrointestinal bleeding, protein-losing enteropathy, or malabsorption. [slideshare.net]

• Myalgia

  Allen JA, Peterson A, Sufit R et al (2011) Post-epidemic eosinophilia-myalgia syndrome associated with L‑tryptophan. [link.springer.com]

  eosinophilia myalgia syndrome (EMS) and toxic oil syndrome (TOS), presented with similar clinical and histopathologic findings as DFE. [the-rheumatologist.org]

  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]

  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]

• Angioedema

  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]

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

  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

  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]

  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]

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

• Eczema

  (b) Brownish eczema-like patches and lichenified changes were noted on the left leg and other extremities. Fig. 2. [medicaljournals.se]

  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]

  Toxins Autoimmune disorders Endocrine disorders Tumors Specific diseases and conditions that can result in blood or tissue eosinophilia include: Acute myelogenous leukemia (AML) Allergies Ascariasis (a roundworm infection) Asthma Atopic dermatitis (eczema [mayoclinic.org]

  Symptoms Symptoms of HES are based on the area affected and may include: Skin: eczema, thickening of the skin, hives, Lungs: cough, shortness of breath, wheezing Heart: chest pain, difficulty breathing, Neurologic disease Blood clots (thrombosis) Eyes [verywellhealth.com]

  […] 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]

• 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]

  In accordance with the remarkable efficacy of dupilumab in alleviating the pruritus associated with atopic dermatitis, as well as its favorable safety profile, we tested its efficacy to decrease our patient's recalcitrant pruritus. [jaadcasereports.org]

  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]

• 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]

  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]

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

  B, Marked improvement in papulation was observed after 3 weeks of treatment with dupilumab. [jaadcasereports.org]

  On the general examination, he had generalized firm non-tender cervical including the preauricular (Figure 2), submandibular, axillary, and inguinal lymphadenopathy with diffuse extensive papules on the trunk relatively sparing the limbs. [cureus.com]

• 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]

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

  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]

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]

While these common causes are considered, one should also proceed with the workup for less common causes of eosinophilia, including autoimmune, idiopathic inflammatory and idiopathic systemic etiologies. [the-rheumatologist.org]

If this workup is unrevealing, idiopathic HES should be considered. [hematologyandoncology.net]

A biopsy confirmed the presence of TNBC, and a full-staging workup did not show any evidence of metastasis. Baseline blood work showed normal complete blood count (CBC) with no evidence of leukocytosis or hypereosinophilia. [jahjournal.org]

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]

Treatment Your treatment will be determined by your type of hypereosinophilic syndrome and severity of your symptoms. It is uncommon to need treatment right at diagnosis, but rarely severe HES may require immediate treatment. [verywellhealth.com]

The treatment was reduced to a 100mg in the third month of treatment due to normalization of eosinophil levels and disappearance of all complaints. The ground-glass nodules disappeared after 1 year of imatinib treatment (Fig. 1b). [journalpulmonology.org]

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

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

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]

Prognosis in hypereosinophilic syndrome[6] Good prognostic factors include response to prednisolone and lack of systemic symptoms. Poor prognosis is associated with anaemia, thrombocytopenia and organ involvement at time of presentation. [patient.info]

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]

*Etiologies specifically discussed in this review A history of travel abroad should raise suspicions for an infectious etiology, because many infections associated with significant eosinophilia are due to helminths. [the-rheumatologist.org]

Therefore, the possible etiologies of secondary eosinophilia were excluded [4]. [cureus.com]

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

Hypereosinophilic Syndrome In this article Introduction Epidemiology History and examination Diagnosis Management Prognosis in hypereosinophilic syndrome See the separate, general article on Eosinophilia. [patient.info]

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]

[…] effective October 01, 2020) D72.119 Hypereosonophilic syndrome (effective October 01, 2020) Signs and Symptoms Angioedema (rapid swelling of the dermis) Cough Diarrhea Fatigue Fever Muscle pains Pruritus Diagnostic Exams Progression and Transformation None Epidemiology [seer.cancer.gov]

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

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

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]

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]

Hypereosinophilic Syndromes: Clinical Manifestations, Pathophysiology, and Diagnosis. (2020). Accessed: January 20, 2022: https://www.uptodate.com/contents/hypereosinophilic-syndromes-clinical-manifestations-pathophysiology-and-diagnosis. [cureus.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]

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]

Because HES can involve multiple organs and have fatal course, we think eosinophil-lowering therapy should be started as soon as possible to prevent aggressive disease progression and organ dysfunction. NOTES References 1. Tefferi A. [e-ce.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]