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]

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]

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]

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]

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]

• Lymphadenopathy

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

  Patients exhibit rapid increases in lymphadenopathy, spleen size, and blood cell numbers, some cells of which take on the appearance of immature and/or malignant cells. [en.wikipedia.org]

  0.36 - 6.3 per 100,000 (J Allergy Clin Immunol 2010;126:179) Previously thought to have male predominance until cases with FIP1L1-PDGFRA fusion were excluded Sites Skin and cardiac involvement common Minority of patients present with splenomegaly and lymphadenopathy [pathologyoutlines.com]

  Physical examination showed erythroderma with extensively infiltrated plaques (Figure 1), excoriated itchy papules (Figure 2), palmoplantar pustules (Figure 3), ear infiltration, periorbital edema, and cutaneous xerosis with multiple axillary lymphadenopathies [cureus.com]

  Patients with L-HES usually present with skin lesions and rheumatologic symptoms and less commonly with lymphadenopathy, gastrointestinal, and pulmonary symptoms. [karger.com]

• Splenomegaly

  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]

  CHIC2 site that causes the FIP1L1/PDGFRA-associated fusion gene (which has tyrosine kinase activity that can transform hematopoietic cells).Patients often have Anemia Elevated serum vitamin B12 levels Hypogranular or vacuolated eosinophils Myelofibrosis Splenomegaly [msdmanuals.com]

  Most patients have anemia, thrombocytopenia, increased serum B12 levels and splenomegaly. [amhsjournal.org]

  Estimated prevalence is 0.36 - 6.3 per 100,000 (J Allergy Clin Immunol 2010;126:179) Previously thought to have male predominance until cases with FIP1L1-PDGFRA fusion were excluded Sites Skin and cardiac involvement common Minority of patients present with splenomegaly [pathologyoutlines.com]

• Cough

  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]

  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]

  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]

  The presenting symptoms of HE and HES are variable and they may include weakness, fatigue, cough, dyspnea and rhinitis, myalgias or angioedema, rash or fever, as well as severe tissue damage or end-organ failure [23][29]. [encyclopedia.pub]

• Dyspnea

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

  The presenting symptoms of HE and HES are variable and they may include weakness, fatigue, cough, dyspnea and rhinitis, myalgias or angioedema, rash or fever, as well as severe tissue damage or end-organ failure [23][29]. [encyclopedia.pub]

  Negative history No history of dyspnea, orthopnea, wheezing, joint pains, oliguria, anuria, worm infestation, or allergic disorders. Drug history The child was initially treated by prednisolone after which he became asymptomatic. [amhsjournal.org]

• 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]

  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]

  pain, vomiting, diarrhea, hepatitis, cholangitis, Budd-Chiari syndrome Cardiac: cardiac damage, valvular fibrosis, thromboembolism Neurologic: behavioral changes, confusion, ataxia, memory loss, peripheral neuropathy Diagnosis Unexplained eosinophilia [pathologyoutlines.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]

  […] 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]

  […] 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]

  • Gastrointestinal symptoms may include abdominal pain, diarrhea, nausea and vomiting. [ojrd.biomedcentral.com]

• Epigastric Pain

  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]

• Early Satiety

  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]

• Periorbital Edema

  On examination, the patient presented with erythroderma, extensive infiltrated plaques, excoriated itchy papules, palmoplantar pustules, ear infiltration, periorbital edema, and cutaneous xerosis. [cureus.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]

  Symptoms related to eosinophilic activation, such as allergy, urticaria/rash, edema, asthma, myalgia/arthralgia, or eosinophil-mediated organ injury, are less frequent in CEL, NOS. [karger.com]

• Joint Stiffness

  TO THE EDITOR: A 26-year-old man presented with a 3-year history of progressive arthralgia and joint stiffness. One year ago, he was examined by a chiropractor for intermittent generalized joint pain and a maculopapular rash on his chest. [bloodresearch.or.kr]

• Angioedema

  A significant proportion of patients with L-HES have episodic angioedema (54%). [frontiersin.org]

  [3][2] The symptom of episodic angioedema (i.e. soft tissue swelling of the face, tongue, larynx, abdomen, arms, or legs) in lymphocyte-variant hypereosinophilia resembles that occurring in Gleich's syndrome, a rare disease that is accompanied by secondary [en.wikipedia.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]

  Patients more often have Angioedema, skin abnormalities, or both Circulating immune complexes (sometimes with serum sickness) Hypergammaglobulinemia (especially high IgE) Patients with the lymphoproliferative variant also more often respond favorably [msdmanuals.com]

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

• Eczema

  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]

  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]

  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]

  Presentation[edit] The typical patient with lymphocyte-variant hypereosinophilia presents with an extended history of hypereosinophilia and cutaneous allergy-like symptoms.[3] Skin symptoms, which occur in >75% of patients, include erythroderma, pruritus, eczema [en.wikipedia.org]

  1319): Dermatologic symptoms were most common, followed by pulmonary, gastrointestinal, cardiac and lastly neurologic 6% of patients presented initially with incidentally detected asymptomatic hypereosinophilia Dermatologic: angioedema, dermographism, eczema [pathologyoutlines.com]

• Pruritus

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

  We report a case of a 52-year-old man with a 12-year history of skin lesions with intense pruritus. [cureus.com]

  [2] Presentation[edit] The typical patient with lymphocyte-variant hypereosinophilia presents with an extended history of hypereosinophilia and cutaneous allergy-like symptoms.[3] Skin symptoms, which occur in >75% of patients, include erythroderma, pruritus [en.wikipedia.org]

  L-HES is a rare, clonal lymphoproliferation of abnormal memory T cells that produces similar clinical symptoms as SS, including severe pruritus and eosinophilia. [oncotarget.com]

  Generalized Recalcitrant Pruritus as the Presenting Manifestation of Hypereosinophilic Syndrome. J Cutan Med Surg. 2016 Jan 29. [QxMD MEDLINE Link]. Roufosse F, Weller PF. Practical approach to the patient with hypereosinophilia. [emedicine.medscape.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]

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

  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]

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

  Typically, the latter had either occasional urticaria, mild facial angioedema, and/or very limited cutaneous lesions. [frontiersin.org]

• Papule

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

  […] such as atopy, food allergy, or seasonal allergy A high peripheral eosinophil count Family history of this condition The endoscopic features are different for different forms, with esophagitis showing strictures and furrows running longitudinally, with papules [news-medical.net]

  We report a case of erythrodermic MF with hypereosinophilic syndrome in a 22-year-old female presenting with gradually progressive intractable erythroderma with intensely pruritic multiple papules, plaques, and nodules involving more than 90% of body [ecancer.org]

  (B) P13, confluent localized papules on abdomen lasting several days. (C,D) P3, disseminated migratory papules on back and arm. (E) P3, large fixed cutaneous plaque lesion on lower leg lasting months. (F) P16, urticaria on thigh, lasting minutes. [frontiersin.org]

• 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 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]

[…] 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]

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

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

Treatment may include prednisone and sometimes imatinib but depends on the specific subtype of hypereosinophilic syndrome. [msdmanuals.com]

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]

Prognosis The prognosis in HES depends on the organ systems involved, disease severity and response to therapy. Outcomes can vary greatly from one person to the next. There is no cure. If HES is left untreated, the disease may be fatal. [apfed.org]

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

Elimination of secondary causes of eosinophilia, evaluation of deep organ involvement, and cytogenetic studies to assess prognosis are paramount. [cureus.com]

Association with hypereosinophilia is important in prognosis. [ecancer.org]

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

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

Although rare, ENKL must also be considered as a potential etiology of HES. 4. [hindawi.com]

Eosinophilia of any etiology can cause the same types of tissue damage. [msdmanuals.com]

Hypereosinophilic syndrome (HES) is a myeloproliferative disorder (MPD) characterized by persistent eosinophilia that is associated with damage to multiple organs. [1, 2, 3, 4, 5, 6, 7] HES is classified into primary (clonal) HES, reactive HES, and (when the etiology [emedicine.medscape.com]

Because HES is a rare neoplasm, its epidemiology has not been accurately investigated. Hence, the disease true incidence is unknown, which is mainly due to the lack of specific coding for the different HES variants. [encyclopedia.pub]

[…] immunophenotypically aberrant T cell population No cytogenetic or molecular abnormalities, however, must exclude clonal hematopoiesis (CHIP) mutations Terminology Idiopathic HES ICD coding ICD-10: D72.110 - idiopathic hypereosinophilic syndrome (IHES) Epidemiology [pathologyoutlines.com]

Epidemiology Frequency Various sources indicate that true idiopathic hypereosinophilic syndrome is rare. [emedicine.medscape.com]

Staphylococcus aureus infections in the early period after lung transplantation: epidemiology, risk factors, and outcomes. J Heart Lung Transplant 2012; 31: 1199–206. CrossRef MEDLINE e92. [aerzteblatt.de]

Epidemiology and clinical features of patients with a CD3−CD4+ T-Cell mediated hypereosinophilic condition. [frontiersin.org]

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

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

Hypereosinophilia (HE) is a heterogeneous condition with a persistent elevated eosinophil count of >350/mm3, which is reported in various (inflammatory, allergic, infectious, or neoplastic) diseases with distinct pathophysiological pathways. [encyclopedia.pub]

Inducible expression and pathophysiologic functions of T-plastin in cutaneous T-cell lymphoma. Blood. 2012; 120:143–54. https://doi.org/10.1182/blood-2011-09-379156. [PubMed]. 38. Gu C, Yao J, Sun P. [oncotarget.com]

CAS Article Google Scholar Roufosse F, Klion A, Weller P: Clinical manifestations, pathophysiology, and diagnosis of the hypereosinophilic syndromes, UpToDate; 2011. http://www.uptodate.com Google Scholar Cogan E, Schandene L, Crusiaux A, Cochaux P, Velu [jmedicalcasereports.biomedcentral.com]

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

The role of NIK in the prevention of Th2 responses is supported further by the fact that gene vaccination using NIK as an adjuvant induces Th1 immune responses (57). [jimmunol.org]

Conclusions HES requires early diagnosis and treatment to prevent fatal complications secondary to deep organ damage. However, the diagnosis is often delayed due to pleomorphic dermatological manifestations. [cureus.com]

Into the tissue, the survival is dependent on local production of cytokines that also prevent eosinophil apoptosis for several days. [encyclopedia.pub]

Long-term treatment with corticosteroids lowers blood eosinophil levels as well as suppresses and prevents complications of the disease in >80% of cases. [en.wikipedia.org]