Idiopathic hypereosinophilicEosinophiliaDiagnosisPlatelet-derivedMyeloproliferative2019PatientsInvolvementMyocarditisSubtypesAcuteImatinibCorticosteroidsGranulomatosisEosinophilAsymptomaticLoeffler'sMultiorganPediatricDisease2022EosinophilsClonalPeripheralManifestationsSeverePresentation2023SymptomsDisordersUncommon2021ReportOnsetDiagnoseInfantChronicRareMastDiagnosticNeutrophilsAtopyInfection
Idiopathic hypereosinophilic13
- Although idiopathic hypereosinophilic syndrome (HES) is uncommon, we studied the clinical characteristics of this disorder in patients with cutaneous involvement. (bmj.com)
- Bain BJ: Eosinophilic leukemias and the idiopathic hypereosinophilic syndrome. (karger.com)
- Idiopathic hypereosinophilic syndrome (IHES) is a rare disease characterized by peripheral blood eosinophilia and eosinophilic infiltration of multiple organs, including the nervous system. (koreamed.org)
- Kwiatkowski V , Navalpakam A., Poowuttikul P. "Idiopathic Hypereosinophilic Syndrome Diagnosed in an Infant: A Case Report. (childrensdmc.org)
- Eventually, a diagnosis of idiopathic hypereosinophilic syndrome was made. (cdc.gov)
- Molecular characterization of the idiopathic hypereosinophilic syndrome (HES) in 35 French patients with normal conventional cytogenetics. (medscape.com)
- Fraticelli P, Kafyeke A, Mattioli M, Martino GP, Murri M, Gabrielli A. Idiopathic hypereosinophilic syndrome presenting with severe vasculitis successfully treated with imatinib. (wjgnet.com)
- Idiopathic hypereosinophilic syndrome (HES) is a rare disorder characterized by peripheral eosinophilia exceeding 1500/mm 3 , a chronic course, absence of secondary causes, and signs and symptoms of eosinophil-mediated tissue injury. (wjgnet.com)
- A thorough understanding of the idiopathic hypereosinophilic syndrome (IHES) and further optimization of diagnostic work-up procedures are warranted. (oncotarget.com)
- Acute exacerbation of idiopathic hypereosinophilic syndrome following asymptomatic coronavirus disease 2019: a case report. (cdc.gov)
- A flare up of idiopathic hypereosinophilic syndrome due to COVID-19. (cdc.gov)
- The idiopathic hypereosinophilic syndrome[J]. Blood, 1994,83:2759-2779. (magtechjournal.com)
- A Case of Idiopathic Hypereosinophilic Syndrome Found in Periodic Health Examination. (kjfm.or.kr)
Eosinophilia17
- This infiltration is necessary for tissue damage to occur because patients with peripheral eosinophilia due to other causes (eg, eosinophilic pneumonia) do not develop pathology similar to hypereosinophilic syndrome. (medscape.com)
- None (0%) of 57 patients with hypereosinophilic syndrome but 10 (56%) of 19 patients with systemic mast cell disease associated with eosinophilia (SMCD-eos) carried the mutated FIP1L1-PDGFRA . (medscape.com)
- Jani K, Kempski HM, Reeves RB: A case of myelodysplasia with eosinophilia having a translocation t(5;12)(q31;q13) restricted to myeloid cells but not involving eosinophils. (karger.com)
- [ 47 ] Also consider terbinafine-induced DRESS (drug reaction with eosinophilia and systemic symptoms) syndrome. (medscape.com)
- Eosinophilia-myalgia syndrome (EMS) was first recognized in 1989 in New Mexico in 3 patients who had an illness with a unique array of symptoms, including peripheral blood eosinophilia and severe myalgias. (medscape.com)
- Also see the Medscape Drugs & Diseases article Eosinophilia-Myalgia Syndrome . (medscape.com)
- Eosinophilia-myalgia syndrome (EMS) is an illness characterized by pruritus, cutaneous lesions, edema, sclerodermoid changes, and joint pain, in addition to dramatic myalgia and eosinophilia. (medscape.com)
- In platelet-derived growth factor receptor alpha (PDGFRA)-associated hypereosinophilic syndrome, eosinophilia is associated with formation of the FLIP1L1/PDFGRA gene, with resultant increased tyrosine kinase activity. (medscape.com)
- One report documents a case of eosinophilia (WBC count, 80,000/μ L with 63% eosinophils) in an infant born to a mother with hypereosinophilic syndrome. (medscape.com)
- Eosinophilia arises either as an intrinsic, clonal disorder or in the majority of cases, secondary to extrinsic conditions, so-called reactive eosinophilia [ 2 - 4 ]. (oncotarget.com)
- One limitation of the traditional definition is that it does not include those patients with some of the same abnormalities (eg, chromosomal defects) that are known causes of hypereosinophilic syndrome but who do not fulfill the traditional hypereosinophilic syndrome definition for degree or duration of eosinophilia. (msdmanuals.com)
- Another limitation is that some patients with eosinophilia and organ damage that characterize hypereosinophilic syndrome require treatment earlier than the 6 months necessary to confirm the traditional diagnostic criteria. (msdmanuals.com)
- General references Hypereosinophilic syndrome is a condition characterized by peripheral blood eosinophilia with manifestations of organ system involvement or dysfunction directly related to eosinophilia in the. (msdmanuals.com)
- The patients have eosinophilia, and lung changes are seen in approximately half of cases. (hrctpatternrecognition.com)
- Diagnosis may sometimes be difficult but it can be achieved with the help of endoscopy plus mucosal biopsies, while peripheral and tissue eosinophilia supported by the findings of radiology, ultrasound and computed tomography can establish the diagnosis in the majority of cases. (openarchives.gr)
- Hypereosinophilic syndrome (HES) constitutes a rare and heterogeneous group of disorders, defined as persistent and marked blood eosinophilia and/or tissue eosinophilia associated with a wide range of clinical manifestations reflecting eosinophil-induced tissue/organ damage. (orpha.net)
- Given the patient's history of recurrent sinusitis, asthma, eosinophilia, and coronary aneurysms, we diagnosed eosinophilic granulomatosis with polyangiitis ( Churg-Strauss syndrome , CSS) and cardiac involvement, and recommended high-dose corticosteroid treatment. (medscape.com)
Diagnosis4
- Eosinophilic colitis and hypereosinophilic syndrome with colic involvement are rare diagnosis that are characterized by wide-ranging gastrointestinal symptoms and idiopathic infiltration of eosinophils in the colon. (karger.com)
- Taking into account the presence of some characteristics that are usually present in cases of reactive HES instead of idiopathic HES, and after an intensive diagnostic study, that could rule out other potential causes of secondary HES, the diagnosis of HES associated with RA was made. (arprheumatology.com)
- That was the case with every child and adult referred with a diagnosis of ADHD. (adhdfraud.net)
- A bone marrow smear was performed, in which 'cerebriform' cells were identified, confirming the diagnosis of Sézary syndrome. (bvsalud.org)
Platelet-derived2
- Fip1-like1-platelet-derived growth factor receptor alpha chain ( FIP1L1-PDGFRA ) mutation has been described in adult patients with hypereosinophilic syndrome. (medscape.com)
- The high efficacy of tyrosine kinase inhibitor, imatinib mesylate (IM), is well-documented in patients with hypereosinophilic syndromes (HES) and detectable rearrangements of platelet-derived growth factor receptor α or β (PDGFRA/B) [1]. (efim.org)
Myeloproliferative1
- Incidence of myeloproliferative hypereosinophilic syndrome in the United States and an estimate of all hypereosinophilic syndrome incidence[J]. J Allergy Clin Immunol,2010, 126:179-181. (magtechjournal.com)
20191
- J Vasc Surg Cases Innov Tech, 2019,5:384-387. (magtechjournal.com)
Patients11
- Also, because some patients with hypereosinophilic syndrome have concomitant neutrophilia, factors other than IL-5 are likely involved. (medscape.com)
- GM-CSF and IL-3 have been shown to be produced by eosinophils, and GM-CSF production was demonstrated in the T-cell clones from patients with hypereosinophilic syndrome. (medscape.com)
- This is a multicenter, open-label, Phase 1/2a dose escalation and expansion study of orally administered emavusertib (CA-4948) monotherapy in adult patients with Acute Myelogenous Leukemia (AML) or high risk Myelodysplastic Syndrome (MDS). (rochester.edu)
- Koga C, Sugita K, Kabashima K, Matsuoka H, Nakamura M, Tokura Y. High responses of peripheral lymphocytes to mosquito salivary gland extracts in patients with Wells syndrome. (medscape.com)
- In patients with cardiac sarcoidosis, giant cell myocarditis or eosinophilic myocarditis the use of immunosuppressive treatment is recommended, as is the case in myocarditis associated with autoimmune disorders. (intechopen.com)
- A detailed anamnesis revealed that both patients consumed unwashed raw vegetables from case-patient 2's garden, which was flooded with runoff from a neighboring cattle pasture in August 2008. (cdc.gov)
- Mepolizumab at the dose of 300 mg/4 weeks has been recently approved as an add-on therapy for patients with uncontrolled hypereosinophilic syndrome (HES) without any identifiable non-hematologic secondary cause. (biomedcentral.com)
- This case illustrates that clinicians should be aware that HES (including severe life-threatening cases) can occur in patients with RA, especially in cases of long-lasting disease with high titters of RF and without treatment, even in the absence of extra-articular features. (arprheumatology.com)
- 76% and 80% of the patients in the case group received anticoagulant therapy or antiplatelet therapy, respectively, 20% received steroid pulse therapy and 64% received immunosuppressive therapy, above which were significantly higher than that in control group( P ＜0.05). (magtechjournal.com)
- Churg-Strauss syndrome (Figure 114) is a small vessel vasculitis occurring in patients with asthma. (hrctpatternrecognition.com)
- Adult patients with hypereosinophilic syndrome (HES) and/or chronic eosinophilic leukemia (CEL) who have FIP1L1-PDGFRα fusion kinase (mutational analysis or FISH demonstration of CHIC2 allele deletion) and for patients with HES and/or CEL who are FIP1L1-PDGFRα fusion kinase negative or unknown. (hikma.com)
Involvement5
- Hypereosinophilic syndrome etiology can involve (1) primitive involvement of myeloid cells, essentially due to the occurrence of an interstitial chromosomal deletion on band 4q12 leading to the creation of the FIP1L1-PDGFRA fusion gene (F/P + variant), or (2) increased interleukin (IL)-5 production by a clonally expanded T-cell population (lymphocytic variant), most frequently characterized by a CD3 - CD4 + phenotype. (medscape.com)
- Cardiac involvement is the most common cause of mortality in hypereosinophilic syndrome. (medscape.com)
- Hypereosinophilic syndrome is a disease characterized by a persistently elevated eosinophil count (≥ 1500 eosinophils/mm³) in the blood for at least six months without any recognizable cause, with involvement of either the heart, nervous system, or bone marrow. (wikipedia.org)
- Hypereosinophilic syndrome with multiorgan involvement: an interdisciplinary work-up. (cdc.gov)
- The IgA level and involvement of skin and peripheral nerves in the case group were significantly higher than that in control group( P ＜0.05). (magtechjournal.com)
Myocarditis2
- In most cases of lymphocytic myocarditis, recovery occurs within a few weeks following supportive treatment. (intechopen.com)
- Transient left ventricular clot in COVID-19-related myocarditis is associated with hypereosinophilic syndrome: a case report. (cdc.gov)
Subtypes1
- subtypes of cutaneous T-cell lymphoma, SS is a malignant neoplasm originating the most common of which are mycosis from T lymphocytes, which involves the fungoides and Sézary syndrome (SS). (bvsalud.org)
Acute3
- Acute eosinophilic pneumonia: A case report and review of the literature. (medscape.com)
- It is recommended:In the case of parasitic infections.In the early stages of Cushing's disease.To diagnose the acute hypereosinophilic syndrome. (orangehealth.in)
- First described in 1998, Paediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal infections (PANDAS) is a specific case of Paediatric Acute-onset Neuropsychiatric Syndrome (PANS) (Quagliarello et al. (isom.ca)
Imatinib3
- Elling C, Erben P, Walz C, Frickenhaus M, Schemionek M, Stehling M. Novel imatinib-sensitive PDGFRA-activating point mutations in hypereosinophilic syndrome induce growth factor independence and leukemia-like disease. (medscape.com)
- Treatment may include prednisone and sometimes imatinib but depends on the specific subtype of hypereosinophilic syndrome. (msdmanuals.com)
- Imatinib mesylate for unmutated hypereosinophilic syndromes: Does it work? (efim.org)
Corticosteroids3
- Many cases of hypereosinophilic syndrome may be treated exclusively with monoclonal antibodies such as mepolizumab It may then be typical not to prescribe corticosteroids for this condition, reducing the burden of the side effects associated with corticosteroids. (wikipedia.org)
- Hypereosinophilic syndrome with isolated Loeffler's endocarditis: complete resolution with corticosteroids. (medscape.com)
- Initial management consisted of en una paciente con topical corticosteroids and oral antihistamines with little clinical response. (bvsalud.org)
Granulomatosis1
- This interdisciplinary grouping of consultants is involved in the management of rare vasculitis and allergic lung conditions such as Churg Straus Syndrome, Wegener's Granulomatosis, Hypereosinophilic syndrome, Anaphylaxis and Immunodeficiency. (tcd.ie)
Eosinophil4
- Eosinophils in hypereosinophilic syndrome infiltrate multiple organs where they inflict tissue damage through the release of granule proteins, including eosinophil peroxidase, major basic protein, eosinophil-derived neurotoxin, and eosinophil cationic protein. (medscape.com)
- Espana A, Sanz ML, Sola J, Gil P. Wells' syndrome (eosinophilic cellulitis): correlation between clinical activity, eosinophil levels, eosinophil cation protein and interleukin-5. (medscape.com)
- In some cases, a slightly elevated eosinophil count may still be within the normal range for that person. (healthchek.in)
- In some cases, a high eosinophil count may be a sign of a more serious condition, such as a blood cancer or a rare autoimmune disease called hypereosinophilic syndrome. (healthchek.in)
Asymptomatic1
- Hypereosinophilic syndrome varies from an asymptomatic phenomenon to a life-threatening multisystem disease. (medscape.com)
Loeffler's3
- Ozdemir O. Loeffler's syndrome: a type of eosinophilic pneumonia mimicking community-acquired pneumonia and asthma that arises from Ascaris lumbricoides in a child. (medscape.com)
- Hypereosinophilic syndrome onset with Loeffler's endocarditis after COVID-19 infection. (cdc.gov)
- Loeffler's syndrome (Figure 111) is characterized by migratory opacities with consolidation which may affect all lobes, both centrally and peripherally. (hrctpatternrecognition.com)
Multiorgan1
- Hypereosinophilic syndrome is a rare multiorgan disease. (hrctpatternrecognition.com)
Pediatric5
- Also see Pediatric Hypereosinophilic Syndrome and Hypereosinophilic Syndrome . (medscape.com)
- Wells syndrome (eosinophilic cellulitis) following vaccination: Two pediatric cases with positive patch test to aluminium salts. (medscape.com)
- Cases of pediatric eosinophilic meningitis following duraplasty with a bovine graft have been reported. (koreamed.org)
- Hypereosinophilic syndrome has a 55.3% male predominance in the pediatric population. (medscape.com)
- James J. Pediatric hypereosinophilic syndrome (HES) differs from adult HES. (medscape.com)
Disease5
- Some possible symptoms a patient may present with include: Cardiomyopathy Skin lesions Thromboembolic disease Pulmonary disease Neuropathy Hepatosplenomegaly Reduced ventricular size Atopic eczema Numerous techniques are used to diagnose hypereosinophilic syndrome, of which the most important is blood testing. (wikipedia.org)
- 3.?Dialogue Hypereosinophilic symptoms (HES) was redefined this year 2010 as a lot more than 1500/mm3 eosinophils with out a discernible extra trigger (eg, HIV disease, worm or parasite infection, allergic illnesses, medication allergies, and nonhematologic malignancies). (rawveronica.com)
- Just as it was my duty to my every patient to diagnose actual disease when it was present, it was equally my duty to make clear to them that they had no disease, when that was the case-when no abnormality could be found. (adhdfraud.net)
- The concept that disease rooted principally in chronic aberrant constitutive and reactive activation of mast cells (MCs), without the gross MC neoplasia in mastocytosis, first emerged in the 1980s, but only in the last decade has recognition of "mast cell activation syndrome" (MCAS) grown significantly. (degruyter.com)
- In some cases, however, an enlarged axillary lymph node may be related to something more serious, such as an infectious disease or cancer. (healthline.com)
20221
- Journal of medical case reports 2022 0 0. (cdc.gov)
Eosinophils1
- Increased production of Interleukin-5 and activated eosinophils has been shown to be the rule in all cases. (openarchives.gr)
Clonal1
- Hypereosinophilic syndrome was previously considered to be idiopathic, but molecular characterization has revealed that many cases have specific clonal disorders. (msdmanuals.com)
Peripheral1
- Methods Among 555 inpatients with HE from Peking Union Medical College Hospital from April 2012 to October 2021, 25 cases complicated with peripheral artery occlusion were involved according to the inclusion and exclusion criteria. (magtechjournal.com)
Manifestations1
- The neurologic manifestations in hypereosinophilic syndrome are. (koreamed.org)
Severe4
- In severe cases, treatment with mechanical circulatory support and/or heart transplantation may be required. (intechopen.com)
- We present a case of a 46 years old woman with long-standing untreated RA, that presented to emergency department with severe symptoms of constrictive pericarditis with cardiac tamponade and bilateral pleural effusion, that progressed to cardiac arrest, associated to symmetrical polyarthritis and pruritic erythematous skin papules. (arprheumatology.com)
- Hypereosinophilic syndrome complicated by severe vascular damage and gangrene. (magtechjournal.com)
- [ii] Approximately 2.5-5% of these cases are characterized as severe. (gsk.com)
Presentation4
- Ghislain PD, Van Eeckhout P. Eosinophilic cellulitis of papulonodular presentation (Wells' syndrome). (medscape.com)
- Holme SA, McHenry P. Nodular presentation of eosinophilic cellulitis (Wells' syndrome). (medscape.com)
- Palisading neutrophilic and granulomatous dermatitis as a presentation of Hodgkin lymphoma: A case and review. (nih.gov)
- Case presentation: The current case study aims to explore the GAPS Nutritional Protocol as a treatment for PANDAS in a 16-years-old patient. (isom.ca)
20231
- Clinical case reports 2023 0 0. (cdc.gov)
Symptoms4
- Case 2 was in the index case's neighbor, who experienced similar symptoms that lasted for 3 months before she was seen at the University Hospital Center Ambroise Paré in Mons in December 2008. (cdc.gov)
- Symptoms of Löffler syndrome are usually mild or absent and tend to spontaneously resolve after several days or, at most, after 2-3 weeks. (medscape.com)
- In low-risk cases of MPNs when people are not experiencing any symptoms, doctors may recommend a watch-and-wait approach rather than beginning treatment immediately. (mympnteam.com)
- Benign cases that don't cause any other symptoms may be treated with a watchful approach only. (healthline.com)
Disorders1
- Contemporary consensus proposal on criteria and classification of eosinophilic disorders and related syndromes[J]. J Allergy Clin Immunol,2012, 130:607-612. (magtechjournal.com)
Uncommon1
- Consumption of watercress and dandelions is uncommon in Belgium, but common in France, where ≈300 HF cases occur annually ( 7 ). (cdc.gov)
20212
Report8
- We report a case of a man admitted to the hospital with a history of nonbloody chronic diarrhea. (karger.com)
- This case report describes the diagnostic workup and highlights the most important features of this often underdiagnosed entity. (karger.com)
- We report 2 cases of human fascioliasis (HF) in Belgium, likely caused by consumption of vegetables from a garden that was flooded by pasture runoff. (cdc.gov)
- A Turkish case report describes a 5-year-old boy with Löffler syndrome caused by A lumbricoides , who initially presented with a 3-day history of cough, dyspnea, wheezing, and intermittent fever. (medscape.com)
- We report here a first case of hypereosinophilia with digestive allergic reaction imputable to docetaxel in a woman treated for breast cancer. (biomedcentral.com)
- This case report was exempt from the Institutional Review Board standards set forth by the Università Politecnica delle Marche. (wjgnet.com)
- Hypereosinophilic Syndrome Following the BNT162b2 (BioNTech/Pfizer) Vaccine Successfully Treated with Mepolizumab: A Case Report and Review of the Literature. (cdc.gov)
- Concurrent hypereosinophilic syndrome and deep vein thrombosis after Pfizer-BioNTech COVID-19 vaccination: A case report. (cdc.gov)
Onset1
- Because autochthonous HF is rare and the route of transmission was unusual, HF was not diagnosed until 6 months after symptom onset in both cases. (cdc.gov)
Diagnose1
- While many cases of enlarged axillary lymph nodes may be temporary or benign, it's important to never self-diagnose any suspicious lump. (healthline.com)
Infant1
- Transient hypereosinophilia in the infant of a mother with hypereosinophilic syndrome. (medscape.com)
Chronic3
- Hypereosinophilic syndrome (HES) and or chronic eosinophilic leukaemia (CEL). (mydr.com.au)
- It can also be used in cases of chronic myeloid leukemia (CML, also called chronic myelogenous leukemia) that have entered the aggressive blast phase - although kinase inhibitors have replaced chemotherapy in most of these cases. (mympnteam.com)
- It may be temporary, such as in the case of an infection, or long term depending on whether it's caused by cancer or a chronic medical condition. (healthline.com)
Rare1
- [ 2 ] Hypereosinophilic syndrome is very rare in children. (medscape.com)
Mast1
- Subsequent literature regarding this newly recognized (but of course not truly new) "mast cell activation syndrome" (MCAS) included case reports as well as formal studies (mostly relatively small scale), reviews, and various proposals for formal diagnostic criteria. (degruyter.com)
Diagnostic2
- Heelan K, Ryan JF, Shear NH, Egan CA. Wells syndrome (eosinophilic cellulitis): Proposed diagnostic criteria and a literature review of the drug-induced variant. (medscape.com)
- The features of our case coincide with the existing diagnostic requirements for HES. (rawveronica.com)
Neutrophils1
- For example, in some cases, the finding of hypersegmented neutrophils on the blood smear was the first hematologic clue to a significant deficiency of vitamin B12 or folate, the erythrocytes lacking the characteristic macrocytosis associated with such deficiencies. (askhematologist.com)
Atopy1
- Wells Syndrome in children and atopy: Retrospective study of 11 cases and review of the literature]. (medscape.com)
Infection3
- A similar timeframe has been described for Löffler syndrome associated with N americanus, A duodenale, or S stercoralis infection. (medscape.com)
- COVID-19 Infection in Hypereosinophilic Syndrome: A survey-based analysis. (cdc.gov)
- Parsonage-turner syndrome associated with SARS-CoV2 (COVID-19) infection. (cdc.gov)