Diversity of clinical manifestations and response to corticosteroids for idiopathic hypereosinophilic syndrome: retrospective study in 33 patients

Helbig, Grzegorz; Wiśniewska-Piąty, Katarzyna; Francuz, Tomasz; Dziaczkowska-Suszek, Joanna; Kyrcz‐Krzemień, Sławomira

doi:10.3109/10428194.2012.731602

Cited by 31 publications

(33 citation statements)

References 15 publications

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“…The study was approved by Local Ethics Committee by Silesian Medical University, Katowice, Poland. Two patients from the current study have been reported elsewhere [5]. …”

Section: Methodsmentioning

confidence: 99%

Characteristics and clinical outcome of patients with hypereosinophilia of undetermined significance

et al. 2013

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The term “hypereosinophilia of undetermined significance” (HEus) previously known as idiopathic, benign eosinophilia relates to patients who have a long-lasting, unexplained and asymptomatic blood HE. These patients have not been studied so far in terms of demographic characteristics and clinical outcome. The aim of this study was to present the clinical characteristics and outcome of HEus patients. This is a retrospective, single-center study of 40 patients with HEus. All patients underwent the basic and specialized evaluations in order to rule out the most common causes of blood HE, but no abnormalities were detected. Twelve patients with at least moderate blood hypereosinophilia (defined as greater than 3.0 × 109/L) for more than 1-year duration were treated with corticosteroids (CS) to avoid end-organ damage. Twenty-one patients (52 %) had an increased leukocyte count at diagnosis. Median blood eosinophilia was 4.2 × 109/L (range 1.5–55.4). HE > 3.0 × 109/L was demonstrated in 17 patients. 65 % of studied population had an increased serum IgE levels, whereas only 2 % demonstrated an increased serum vitamin B12 levels. A median bone marrow infiltration by eosinophils was 30.5 % (range 11–78.2). All treated patients responded promptly to CS and remained in complete remission while receiving low doses of CS (20 mg/day to 5 mg every 2-day). One patient developed hypereosinophilic syndrome (HES) after 11 years of follow-up. Further studies are needed to define risk factors of HES development. The use of CS for HEus patients is controversial and should be individualized.

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“…The study was approved by Local Ethics Committee by Silesian Medical University, Katowice, Poland. Two patients from the current study have been reported elsewhere [5]. …”

Section: Methodsmentioning

confidence: 99%

Characteristics and clinical outcome of patients with hypereosinophilia of undetermined significance

et al. 2013

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“…The management of cardiac disease in HES is to normalise the damaging HE. Corticosteroids are one of the cornerstones of therapy for HES to reduce eosinophil count and counteract inflammation 36. Patients with the FIP1L1-PDGFRA mutation should be treated with a tyrosine kinase inhibitor (imatinib) given its possible effectiveness37 along with steroids (with cardiac involvement, steroids should be used along with the imatinib to prevent the occurrence of acute heart failure from necrotising myocarditis) 38.…”

Section: Management Of Hes Cardiac Diseasementioning

confidence: 99%

Hypereosinophilic syndrome: cardiac diagnosis and management

Mankad

Bonnichsen

Mankad

2015

Heart

120

158

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Hypereosinophilic syndrome (HES) is a heterogeneous group of conditions that is defined at its core by hypereosinophilia (HE) (blood eosinophil count of >1.5×10(9)/L) and organ damage directly attributable to the HE. Cardiac dysfunction occurs frequently in all forms of HES and is a major cause of morbidity and mortality. Once a significantly elevated eosinophil count is identified, it must be confirmed on repeat testing and the aetiology for the HE must be rigorously sought out with a focus on identifying whether organ dysfunction is occurring. Echocardiography is routinely performed to assess for cardiac involvement, looking for evidence of left ventricular and/or right ventricular apical obliteration or thrombi or a restrictive cardiomyopathy. Cardiac magnetic resonance imaging and CT are often useful adjuncts to establish the diagnosis but endomyocardial biopsy remains the gold standard. To decrease the degree of eosinophilia, treatment can include corticosteroids and/or imatinib based on the aetiology. Anticoagulation, standard heart failure therapy for a restrictive cardiomyopathy and finally cardiac transplantation may be indicated in the treatment algorithm.

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“…Among patients treated with 30–40 mg of prednisone daily, with subsequent tapering to a maintenance dose, objective response rates range from 65%–85%. 49,50 Disease progression while on prednisone doses >10 mg daily warrants the addition of a second agent. Hydroxyurea has been used alone or together with corticosteroids in previously untreated or steroid-refractory HES patients with response rates around 70%.…”

Section: Eosinophilic Myeloid Disordersmentioning

confidence: 99%

Eosinophilia in Hematologic Disorders

Falchi¹,

Verstovšek²

2015

Immunology and Allergy Clinics of North America

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Synopsis A finding of eosinophilia in the peripheral blood can be the manifestation of a large number of different medical conditions, including benign or malignant disorders. From a diagnostic standpoint eosinophilia can be divided into reactive (secondary) or clonal (primary). There are three main types of WHO-defined eosinophilia-associated myeloid neoplasms (MN-eos): 1) myeloid and lymphoid neoplasms associated with rearrangements of PDGFRA, PDGFRB or FGFR1; 2) chronic eosinophilic leukemia, not otherwise specified (CEL-NOS); and 3) idiopathic hypereosinophilic syndrome (HES). Imatinib mesylate, a PDGFRA and PDGFRB inhibitor, has revolutionized the treatment of molecularly defined MN-eos. Second generation molecules are available for patients who fail imatinib. Novel agents, such as the anti-IL5 antibody mepolizumab, have been successfully used for the treatment of HES. The discovery of new, recurrent molecular alterations in patients with MN-eos may improve the diagnosis and therapy of this group of patients. This review focuses on the hematologist’s approach to a patient with eosinophilia as well as treatment options for patients with eosinophilic myeloid neoplasms.

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Diversity of clinical manifestations and response to corticosteroids for idiopathic hypereosinophilic syndrome: retrospective study in 33 patients

Cited by 31 publications

References 15 publications

Characteristics and clinical outcome of patients with hypereosinophilia of undetermined significance

Characteristics and clinical outcome of patients with hypereosinophilia of undetermined significance

Hypereosinophilic syndrome: cardiac diagnosis and management

Eosinophilia in Hematologic Disorders

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