EosinophilsEosinophilic granulomatosis with polyangiitisHeterogeneous group of disordersDiagnosisPeripheral bloodFIP1L1-PDGFRATyrosineEosinophil countMyeloid leukemiaDisordersImatinibClonal1500CharacterizeHematologicAcuteMyocardialSystemicDiseasePatientsSevereSecondaryClinicalPersistentIncidenceDisorderMagneticPrimaryTreatmentBloodDiagnosticUnknownConditionMildDiarrheaAbsenceCasesCaseMedical
Eosinophils2
- These causes are classified as primary (i.e. a defect intrinsic to the eosinophil cell line), secondary (induced by an underlying disorder that stimulates the proliferation and activation of eosinophils), or idiopathic (i.e. unknown cause). (wikipedia.org)
- 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)
Eosinophilic granulomatosis with polyangiitis1
- Differential diagnoses include drug allergies and parasitic infections, solid and hematological malignancies (i.e. chronic myeloid leukemia), eosinophilic granulomatosis with polyangiitis and human T cell lymphotropic virus infection (see these terms). (orpha.net)
Heterogeneous group of disorders1
- 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)
Diagnosis2
- This manuscript discusses the recommendations outlined in the NCCN Guidelines for the diagnosis and management of patients with chronic phase-CML. (bvsalud.org)
- Therefore, the diagnosis of autoimmune hepatitis-primary sclerosing cholangitis overlap syndrome was made, and prednisolone, azathioprine, and ursodeoxycholic acid was initiated. (biomedcentral.com)
Peripheral blood3
- Hypereosinophilia has generally been defined as a peripheral blood eosinophil count greater than 1500/μL. (medscape.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)
- Peripheral blood hypereosinophilia in conjunction with autoimmune hepatitis-primary sclerosing cholangitis overlap syndrome has not been reported yet. (biomedcentral.com)
FIP1L1-PDGFRA5
- An interstitial deletion del(4)(q12q12) generating a FIP1L1-PDGFRA fusion gene is observed in diverse eosinophilia-associated hematologic disorders like hyperseosinophilic syndrome (HES), systemic mastocytosis (SM) and chronic eosinophilic leukemia (CEL). (atlasgeneticsoncology.org)
- The cause of FIP1L1-PDGFRA associated hypereosinophilic syndrome is unknown as well as its association with predominantly male sex. (atlasgeneticsoncology.org)
- Data support a FIP1L1-PDGFRA fusion incidence of approximately 10-20% among patients presenting with idiopathic hypereosinophilia (Gotlib and Cools, 2008). (atlasgeneticsoncology.org)
- FIP1L1-PDGFRA associated hypereosinophilic disorders are sensitive to treatments with tyrosine kinase inhibitors such as imatinib mesylate (imatinib). (atlasgeneticsoncology.org)
- however chronic eosinophilic leukemia with FIP1L1-PDGFRA is likely to be responsive also to dasatinib, nilotinib, sorafenib and midostaurin (PKC412) (Lierman et al. (atlasgeneticsoncology.org)
Tyrosine2
- Patients with hypereosinophilic syndrome historically carried a poor prognosis before the successful therapeutic application of tyrosine kinase inhibitors. (atlasgeneticsoncology.org)
- Tyrosine kinase inhibitor (TKI) therapy is a highly effective treatment option for patients with chronic phase-CML. (bvsalud.org)
Eosinophil count1
- We investigated the efficacy and safety of mepolizumab 100 mg in idiopathic HES (I-HES) patients as a steroid sparing strategy for disease remission maintenance by assessing clinical conditions, blood eosinophil count (BEC) and adverse events at baseline and at 3-6-12 months follow-up. (biomedcentral.com)
Myeloid leukemia1
- Interestingly, the T674I mutation that is analogous to the T315I mutation of BCR-ABL1 in chronic myeloid leukemia also confers imatinib resistance (Cools et al. (atlasgeneticsoncology.org)
Disorders5
- Increased levels of eosinophilic leukocytes in the blood can be idiopathic, or may result from a variety of conditions, including connective tissue diseases, helminthic infections, neoplasias, and allergic disorders. (medscape.com)
- The purpose of this study is to characterize the immunophenotype of individuals with hypereosinophilia-associated cardiovascular, gastrointestinal, hematologic, and respiratory disorders. (mayo.edu)
- Hypereosinophilic syndrome was previously considered to be idiopathic, but molecular characterization has revealed that many cases have specific clonal disorders. (msdmanuals.com)
- The WAS-related disorders, which include Wiskott-Aldrich syndrome, X-linked thrombocytopenia (XLT), and X-linked congenital neutropenia (XLN), are a spectrum of disorders of hematopoietic cells, with predominant defects of platelets and lymphocytes caused by pathogenic variants in WAS. (beds.ac.uk)
- Contemporary consensus proposal on criteria and classification of eosinophilic disorders and related syndromes[J]. J Allergy Clin Immunol,2012, 130:607-612. (magtechjournal.com)
Imatinib2
- 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)
- Treatment may include prednisone and sometimes imatinib but depends on the specific subtype of hypereosinophilic syndrome. (msdmanuals.com)
Clonal2
- Primary eosinophilia is not a reactive phenomenon and can be described as either clonal or idiopathic in nature. (medscape.com)
- If reactive causes are ruled out and no underlying clonal origin is proven, the eosinophilia is described as idiopathic. (medscape.com)
15001
- 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)
Characterize1
- 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)
Hematologic1
- 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)
Acute1
- it is dominated by symptoms characterized as the acute coronary syndrome such as angina, heart attack and/or congestive heart failure. (wikipedia.org)
Myocardial1
- Hypereosinophilia (i.e. blood eosinophil counts at or above 1,500 per microliter) or, less commonly, eosinophilia (counts above 500 but below 1,500 per microliter) are found in the vast majority of cases of eosinophilic myocarditis and are valuable clues that point to this rather than other types of myocarditis or myocardial injuries. (wikipedia.org)
Systemic1
- We believe that cysteinyl leukotriene type 1 receptor antagonists are safe and effective drugs for most patients with asthma but caution is needed for those with more severe disease who require systemic corticosteroids, especially if they show characteristics of the atypical allergic diathesis seen in the prodromal phase of Churg-Strauss syndrome. (lookformedical.com)
Disease3
- The primary goal of TKI therapy in patients with chronic phase-CML is to prevent disease progression to accelerated phase-CML or blast phase-CML. (bvsalud.org)
- Whole-exome sequencing and genome-wide methylation analyses identify novel disease associated mutations and methylation patterns in idiopathic. (oncotarget.com)
- In true idiopathic RCM, endomyocardial biopsy and pathologic specimen findings are usually abnormal, although they may not be diagnostic for any single disease. (medscape.com)
Patients4
- Chronic MCL (lack of C-findings) comprised 14% of patients, and only 4.5% had "leukemic MCL" (≥10% circulating MCs). (bvsalud.org)
- 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)
- Analysis of clinical characteristics of 25 patients with hypereosinophilia complicated with peripheral arterial occlusion[J]. Basic & Clinical Medicine, 2023, 43(5): 785-789. (magtechjournal.com)
- We assessed the clinicopathological features of 28 patients with peripheral neuropathy associated with Churg-Strauss syndrome. (lookformedical.com)
Severe1
- Hypereosinophilic syndrome complicated by severe vascular damage and gangrene. (magtechjournal.com)
Secondary1
- Viral and autoimmune hepatitis were excluded, and secondary workups for hypereosinophilia were all negative. (biomedcentral.com)
Clinical2
- Hypereosinophilia with concurrent venous thromboembolism: clinical features, potential risk factors, and short-term outcomes in a Chinese cohort[J]. Sci Rep, 2020,10:8359. (magtechjournal.com)
- Autoimmune hepatitis-primary sclerosing cholangitis (AIH-PSC) overlap syndrome is a clinical, biochemical, immunological, and histological feature of AIH with cholangiographic abnormalities characteristic of PSC [ 1 ]. (biomedcentral.com)
Persistent1
- In those with unexplained persistent asymptomatic hypereosinophilia (HE), the provisional term HE of undetermined significance is given. (orpha.net)
Incidence1
- 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)
Disorder1
- Typically, the disorder is associated with hypereosinophilia, i.e. an eosinophil blood cell count greater than 1,500 per microliter (normal 100 to 400 per microliter). (wikipedia.org)
Magnetic1
- Autoimmune hepatitis-primary sclerosing cholangitis overlap syndrome is a form of autoimmune hepatitis with cholestatic features and is characterized by negative anti-mitochondrial antibody and cholangiographic changes on magnetic resonance cholangiopancreatography or endoscopic retrograde cholangiopancreatography. (biomedcentral.com)
Primary1
- Here we present a case of autoimmune hepatitis-primary sclerosing cholangitis overlap syndrome with hypereosinophilia. (biomedcentral.com)
Treatment1
- Therapy for idiopathic restrictive cardiomyopathy (RCM) is limited to symptomatic treatment and is often ineffective in improving outcome. (medscape.com)
Blood1
- The idiopathic hypereosinophilic syndrome[J]. Blood, 1994,83:2759-2779. (magtechjournal.com)
Diagnostic1
- A thorough understanding of the idiopathic hypereosinophilic syndrome (IHES) and further optimization of diagnostic work-up procedures are warranted. (oncotarget.com)
Unknown1
- However, in roughly 3/4 of cases, pathogenesis remains unknown, now defining idiopathic HES. (orpha.net)
Condition1
- PDGFRA-associated chronic eosinophilic leukemia is often grouped with a related condition called hypereosinophilic syndrome. (beds.ac.uk)
Mild1
- other complications of Wiskott-Aldrich syndrome, including eczema and immune dysfunction, are usually mild or absent. (beds.ac.uk)
Diarrhea1
- We report a case of a man admitted to the hospital with a history of nonbloody chronic diarrhea. (karger.com)
Absence1
- Thrombocytopenia absent radius (TAR) syndrome is characterized by bilateral absence of the radii with the presence of both thumbs, and thrombocytopenia that is generally transient. (beds.ac.uk)
Cases2
- Most cases of RCM (including idiopathic ones) are not known to be inherited, although there have been reports of families in whom multiple members are affected by a combination of hypertrophic and restrictive cardiomyopathies. (medscape.com)
- Several cases of eosinophilic conditions including Churg-Strauss syndrome have been reported to be associated with zafirlukast, a cysteinyl leukotriene type 1 receptor antagonist. (lookformedical.com)
Case1
- Here we present a case of AIH-PSC overlap syndrome with hypereosinophilia. (biomedcentral.com)
Medical1
- Decades of research suggest that Mindfulness-Based Stress Reduction (MBSR) training supports a greater capacity to live with chronic medical conditions and contributes to lowering stress levels. (preprints.org)