ABSTRACTS: ACCEPTED: COLON—CLINICAL VIGNETTES/CASE REPORTS S1918 Lymphocytic Variant of Hypereosinophilic Syndrome Presenting with Gastroenteritis 1Weill Cornell Medicine - Qatar, New York, NY; 2New York Presbyterian - Weill Cornell Medical Center, New [journals.lww.com]

(L-HES) is a potentially fatal disease that requires timely diagnosis, but the presentation in children is not well characterized. [aaaai.confex.com]

Case Presentation A 52-year-old male presented with a three-year history of periodic pruritic and generalized erythema that had been diagnosed as mycosis fungoid, which was suspected in a single histological study and whose immunohistochemical study did [cureus.com]

CASE PRESENTATION: A 42 year old male with a past medical history of mild-moderate asthma presented to the intensive care unit for further management of multifocal pneumonia. [journal.chestnet.org]

Image of the palms at 2 years after presentation showing papules and pustules on the palmar surface of left hand. Seven years after presentation, the patient's eosinophil count increased to 2.1 × 109/L. [ncbi.nlm.nih.gov]

• Lymphadenopathy

  We present a rare case of idiopathic HES presenting with lymphadenopathy in a young child. [amhsjournal.org]

  2020 / Accepted: 27 August 2020 / Published: 29 August 2020 Abstract Sézary syndrome (SS), an aggressive cutaneous T-cell lymphoma (CTCL) with poor prognosis, is characterized by the clinical hallmarks of circulating malignant T cells, erythroderma and lymphadenopathy [mdpi.com]

  Most patients with SLL present with indolent generalized lymphadenopathy, which has frequently been present for several years. [jahjournal.org]

  There was no lymphadenopathy or organomegaly. Skin biopsy showed superficial perivascular dermatitis with vascular thrombosis and eosinophilia that was nondiagnostic. [ncbi.nlm.nih.gov]

  Under treatment, there was rapid remission of lymphadenopathy, but the OCS dose could not be reduced below 7.5 mg/day to achieve symptom freedom. [link.springer.com]

• 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]

  Gastrointestinal tract - tract inflammation, infarction of the gut secondary to emboli, splenomegaly, ascites, hepatitis, pancreatitis. Neurological - acute cerebrovascular accident, confusion, ataxia, peripheral neuropathy. [patient.info]

  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]

  […] patients; c) episodic angioedema-related gastrointestinal symptoms that are sometimes similar to symptoms of the irritable bowel syndrome occurring in ~20% of patients; d) rheumatologic manifestations of inflammatory arthralgias in ~20% of patients; and e) splenomegaly [en.wikipedia.org]

• Cough

  Results: A previously-healthy 15 year-old boy developed recurrent episodes of dyspnea, cough, and wheezing. He was diagnosed with asthma and despite high-dose inhaled corticosteroids he required ten hospitalizations for asthma exacerbation. [aaaai.confex.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]

  He had recently seen his primary care physician four months prior to admission with a productive cough and bilateral lower lobe opacities on chest Xray. [journal.chestnet.org]

  Individuals with idiopathic hypereosinophilic syndrome may experience symptoms such as: weight loss fevers night sweats fatigue coughing difficulty breathing abdominal pain vomiting and diarrhea muscle aches joint pain chest pain swelling stomach ache [medicalnewstoday.com]

  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 by involvement of lungs (cough [orpha.net]

• Dyspnea

  Results: A previously-healthy 15 year-old boy developed recurrent episodes of dyspnea, cough, and wheezing. He was diagnosed with asthma and despite high-dose inhaled corticosteroids he required ten hospitalizations for asthma exacerbation. [aaaai.confex.com]

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

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

  […] pulmonary, gastrointestinal, cardiac and lastly neurologic 6% of patients presented initially with incidentally detected asymptomatic hypereosinophilia Dermatologic: angioedema, dermographism, eczema, erythroderma, pruritis, urticaria Pulmonary: cough, dyspnea [pathologyoutlines.com]

  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]

• Abdominal Pain

  During the active phases of the disease, the patient with IBD has frequent discharges of diarrhoea, including bloody diarrhoea, abdominal pain, weight loss and fever. [emergency-live.com]

  At age 16 he presented with respiratory distress, hemoptysis, and abdominal pain. Absolute eosinophil count (AEC) was 10636 (57.9%), peaked at 30377 and IgE level 11600; one month prior, AEC 49 (0.5%) and total IgE 4070. [aaaai.confex.com]

  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]

• Diarrhea

  He then began having severe epigastric pain, early satiety, a 20-pound weight loss, and his diarrhea recurred. He was found to have a hemoglobin of 6.4 and an elevated absolute eosinophil count of 1.35 x 103 u/L. [journals.lww.com]

  The mucosal form of eosinophilic gastrointestinal disorder is the most common type and shows up with abdominal cramps, nausea, vomiting, indigestion, diarrhea, melena, and weight loss with protein loss. [news-medical.net]

  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]

  In the first category, symptoms included: fever greater than 37.5 degrees Celsius, apnea, cough, tachypnea, respiratory distress or recession, supplemental oxygen requirement, and vomiting or diarrhea. [contemporarypediatrics.com]

• Vascular Disease

  (Löffler syndrome), cyclical eosinophilia, skin diseases (angiolymphoid hyperplasia), collagen vascular diseases such as Churg-Strauss syndrome and Kimura disease Myeloproliferative variants of HES (M-HES): HES with features of myeloproliferative disorder [pathologyoutlines.com]

  Eosinophilia has been confirmed to result in damage to the vascular wall, which in turn can cause vascular disease.1 Peak absolute eosinophil count in peripheral blood was found to be an independent risk variable for pulmonary embolism in patients with [dovepress.com]

  The causes include infection, allergy (especially bronchopulmonary aspergillosis), toxins, and collagen vascular disease. Some are idiopathic. [karger.com]

• Arthralgia

  On admission, he complained of fatigue, night sweats, and arthralgia. His physical examination showed unremarkable results except for a few skin nodules on his left leg. [bloodresearch.or.kr]

  […] pneumonia, occurring in ~20% of patients; c) episodic angioedema-related gastrointestinal symptoms that are sometimes similar to symptoms of the irritable bowel syndrome occurring in ~20% of patients; d) rheumatologic manifestations of inflammatory arthralgias [en.wikipedia.org]

• 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]

  Non-episodic Angioedema With Eosinophilia Successfully Treated With Reslizumab. Allergy Asthma Immunol Res. 2020;12(2):371-4. 6. Walsh GM. [ijaai.tums.ac.ir]

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

  Since the patient showed clinical manifestations of L-HES as well as episodic angioedema with eosinophilia (EAE), a continuous periodic examination is required given the higher risk of developing lymphoma or leukemia. [physiciansweekly.com]

• Eczema

  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]

  There was no history of allergy i.e. any history of hay - fever, eczema, bronchial asthama etc. Family history was also insignificant. She had history of reccurence of symptoms whenever she stopped steroid therapy. [ijdvl.com]

  From a dermatological perspective, it is necessary to look for dermatological lesions suggestive of a specific dermatological pathology with histopathological confirmation such as autoimmune bullous dermatoses, eczema, or atopic dermatitis [5]. [cureus.com]

• Pruritus

  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]

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

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

  The skin lesions are particularly prevalent in L-HES and are commonly associated with pruritus. Eczema, erythroderma, urticaria and angioedema along with lymphadenopathy are common in this type. [dermatologyadvisor.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]

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

  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]

• Papule

  We cite, for example, pruritic erythematous papules, urticaria, angioedema, dermographism, mouth and genital ulcers, erythema annulare centrifugum, acral bullae, and erythroderma. [cureus.com]

  Subsequently, she experienced a flare of symptoms involving erythematous papules on her lower back and arms. She restarted MFA, and her skin cleared within several weeks. [ncbi.nlm.nih.gov]

  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]

  When cutaneous lesions appear as erythematous, papules, and blisters, the differential diagnosis of persistent herpes zoster should be considered. [dovepress.com]

• Stroke

  Neurologic Complication • The first type : Embolic strokes • The second type : Encephalopathy : Upper motor neuron signs • The third type : Peripheral neuropathy Klion AD et al.Middleton's Allergy ; 8th edition. 2014. p. 1205-23. 87. [slideshare.net]

  Stroke or transient ischemic episodes may occur following peripheral embolism of intracardiac thrombi. [ojrd.biomedcentral.com]

The remaining three had an unknown diagnosis due to lack of information and additional workup would be warranted. [mayoclinic.pure.elsevier.com]

The eosinophilia did not completely resolve and a bone marrow aspirate did not show clonal eosinophilic disease or malignancy and workup was negative for autoimmune, parasitic, or other infectious disease. [journal.chestnet.org]

After undergoing a standard workup he was diagnosed with rheumatoid arthritis and given low-dose methotrexate, which was switched to leflunomide as his symptoms did not improve. He was referred to our clinic because of eosinophilia. [bloodresearch.or.kr]

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

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]

• Neutrophilia

  Investigations[3, 6] FBC - eosinophils >1.5 x 109/L (0.5 x 109/L under the new definition); neutrophilia and anaemia are also common. Platelet counts can be high or low. [patient.info]

  However, the term “eosinophilic variant CML” [30, 31] is specifically used to describe a variant of CML with prominent eosinophilia with no significant neutrophilia or basophilia. [karger.com]

  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]

  Persistent neutrophilia is a marker for an increased risk of venous thrombosis. J Thromb Thrombolysis. 2016;42(4):545–551. doi:10.1007/s11239-016-1398-4 17. Esmaeilzadeh H, Nouri F, Nabavizadeh SH, et al. [dovepress.com]

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

Since reslizumab treatment was initiated, her eosinophil count remained consistently within the normal range. [ijaai.tums.ac.ir]

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]

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]

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 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]

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]

HES has been appreciated for some time, the recent characterization of some of these variants at the molecular and immunologic levels has demonstrated dramatic differences in disease pathogenesis, response to treatment, and prognosis depending on the etiology [semanticscholar.org]

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

When the cutaneous manifestations do not allow a diagnosis, the etiological investigation must be systematic. [cureus.com]

Etiology Recent advances in underlying pathogenesis have established that what was once thought to be ''idiopathic'' HES may in some cases be due to either primitive involvement of myeloid cells (primary HES), essentially due to occurrence of an interstitial [orpha.net]

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]

The data on patients’ characteristics, epidemiology, clinical features, diagnosis, treatment and outcome were collected and analysed. [casereports.bmj.com]

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

Epidemiology of neonatal COVID-19 in the United States. Pediatrics. 2022;150(4). doi:10.1542/peds.2022-056297 [contemporarypediatrics.com]

Potential roles of eosinophils in cancer therapy: epidemiological studies, experimental models, and clinical pathology. Cao C, Gu Y, Zhu C, Palmai-Pallag T, Lan F, Chen Z, Li W, Shen H, Ying S. Cao C, et al. [pubmed.ncbi.nlm.nih.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]

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

Neuroimmune destabilization in the VZV-infected site may be mediated by the continuous influence of viral DNA.4 The definitive pathophysiologic mechanisms of chronic GVHD are complex. [dovepress.com]

The molecular characterization of this subset of patients responding to imatinib may contribute to an understanding of the pathophysiology of these rare disorders and suggest new therapeutic targets. [ascopubs.org]

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 of colon cancer As a secondary prevention of colon cancer risk, it is very useful to regularly perform a chromoendoscopy, i.e. an endoscopy with dye that can highlight any areas of dysplasia and/or neoplasia. [emergency-live.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]