Interferon-α in the idiopathic hypereosinophilic syndrome: consideration of five cases

Ceretelli, Silvia; Capochiani, Enrico; Petrini, Mario

doi:10.1007/s002770050434

Cited by 60 publications

(34 citation statements)

References 10 publications

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“…The end points of this study were based on data that showed that CML patients receiving imatinib therapy who underwent monitoring of the BCR-ABL transcript burden by real-time quantitative polymerase chain reaction and had a reduction of 3log 10 or more of BCR-ABL transcripts had a negligible risk of disease progression. 75 Lim et al 76 Myeloproliferative neoplasms Polycythemia vera and essential thrombocythemia Single agent Quintas-Cardama et al, 46 Kiladjian et al 78 Hypereosinophilic syndrome Resistance to steroids, hydroxyurea, and TKI-insensitive Single agent Butterfield and Gleich, 95 Ceretelli et al 96 …”

Section: Combination Therapy With Ifn␣ and Imatinib In CMLmentioning

confidence: 99%

The renaissance of interferon therapy for the treatment of myeloid malignancies

Kiladjian

Mesa

Hoffman

2011

Blood

179

144

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Section: Combination Therapy With Ifn␣ and Imatinib In CMLmentioning

confidence: 99%

The renaissance of interferon therapy for the treatment of myeloid malignancies

Kiladjian

Mesa

Hoffman

2011

Blood

179

144

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“…138,139 Interferon-␣ (IFN-␣) can elicit long-term hematologic and cytogenetic responses in HES and CEL patients resistant to other therapies, including prednisone and hydroxyurea. 109,[140][141][142][143][144][145] Some have advocated its use as initial therapy for these diseases. 144 Remissions have been associated with improvement in clinical symptoms and organ disease, including hepatosplenomegaly, 140,144 cardiac and thromboembolic complications, 109,141 mucosal ulcers, 143 and skin involvement.…”

Section: Current Treatmentmentioning

confidence: 99%

“…109,[140][141][142][143][144][145] Some have advocated its use as initial therapy for these diseases. 144 Remissions have been associated with improvement in clinical symptoms and organ disease, including hepatosplenomegaly, 140,144 cardiac and thromboembolic complications, 109,141 mucosal ulcers, 143 and skin involvement. 145 IFN-␣ exerts pleiotropic effects including inhibition of eosinophil proliferation and differentiation.…”

Section: Current Treatmentmentioning

confidence: 99%

The FIP1L1-PDGFRα fusion tyrosine kinase in hypereosinophilic syndrome and chronic eosinophilic leukemia: implications for diagnosis, classification, and management

Gotlib

Cools

Malone

et al. 2004

Blood

262

205

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Idiopathic hypereosinophilic syndrome (HES) and chronic eosinophilic leukemia (CEL) comprise a spectrum of indolent to aggressive diseases characterized by unexplained, persistent hypereosinophilia. These disorders have eluded a unique molecular explanation, and therapy has primarily been oriented toward palliation of symptoms related to organ involvement. Recent reports indicate that HES and CEL are imatinib-responsive malignancies, with rapid and complete hematologic remissions observed at lower doses than used in chronic myelogenous leukemia (CML). These BCR-ABL-negative cases lack activating mutations or abnormal fusions involving other known target genes of imatinib, implicating a novel tyrosine kinase in their pathogenesis. A bedside-to-benchtop translational research effort led to the identification of a constitutively activated fusion tyrosine kinase on chromosome 4q12, derived from an interstitial deletion, that fuses the platelet-derived growth factor receptor-␣ gene (PDGFRA) to an uncharacterized human gene FIP1-like-1 (FIP1L1). However, not all HES and CEL patients respond to imatinib, suggesting disease heterogeneity. Furthermore, approximately 40% of responding patients lack the FIP1L1-PDGFRA fusion, suggesting genetic heterogeneity. This review examines the current state of knowledge of HES and CEL and the implications of the FIP1L1-PDGFRA discovery on their diagnosis, classification, and management. IntroductionProtean biologic and clinical presentations characterize idiopathic hypereosinophilia (HES). HES is similar to other diseases given the moniker "diagnosis of exclusion," in that limited understanding of the pathogenesis of the disease has hampered therapeutic advances. The demonstration of increased myeloblasts or clonality or the development of either granulocytic sarcoma or acute myeloid leukemia helps clarify the origin of some cases of chronic eosinophilic leukemia. 1 In a subset of patients, hypereosinophilia is related to excessive secretion of eosinophilopoietic cytokines from a clonal population of lymphocytes. 2 The identification of FIP1-like-1-platelet-derived growth factor receptor-␣ (FIP1L1-PDGFRA) in cases of HES/CEL adds to a growing list of activated fusion tyrosine kinases linked to the pathogenesis of chronic myeloproliferative disorders. 3 It is unique, however, because it is the first description of a gain-of-function fusion protein resulting from a cryptic interstitial deletion between genes rather than a reciprocal chromosomal translocation. The FIP1L1-PDGFR␣ fusion protein transforms hematopoietic cells, and its kinase activity is inhibited by imatinib at a cellular 50% inhibitory concentration (IC 50 ) 100-fold lower than BCR-ABL. 3 Acquisition of an imatinib resistance mutation in the adenosine triphosphate (ATP)-binding domain of PDGFRA in a relapsed patient previously responsive to imatinib supports a critical role for FIP1L1-PDGFR␣ in the pathogenesis of disease and demonstrates that FIP1L1-PDGFR␣ is the therapeutic target of imatinib. 3 The identification of t...

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“…In this case, prednisolone therapy was very effective, and the patient's symptoms dramatically disappeared. Some investigators have reported that IFN-therapy is effective in steroid-resistant cases (14,15). When symptoms recur in a patient, another therapy must be chosen.…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Idiopathic Hypereosinophilic Syndrome Involving the Oral Cavity Associated with the Esophagus and Gastrointestinal Tract

et al. 2004

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We report the rare case of HES involving oral cavity associated with esophagus, and gastrointestinal tract, which we succeeded in diagnosing precisely through a biopsy specimen obtained from the lip. A 64-year-old man had dysphagia, swelling of the oral mucosa and the posterior cervical muscles, accompanied by an abdominal pain and diarrhea. Peripheral blood cell count showed marked eosinophilia. Case ReportA 64-year-old man had a 9-day history of dysphagia and swelling of the oral mucosa and posterior cervical muscles. Also he had abdominal pain and diarrhea for 4 days.He had bronchial asthma for the previous 1 year, which was controlled with regular inhalations of budesonide and oral theophyline and occasional inhalations of salbutamol. There were no recent infections or relevant travel history.At admission, he was unwell with a low-grade fever up to 37.1°C, swelling of oral mucosa (Fig. 1), and frequent bowel movement. Because of dysphagia and severe swelling of the oral mucosa, he could not eat any food.Laboratory examinations revealed an excess of white blood cells, 17,800/mm , with marked eosinophilia, and 52.7% eosinophils. Serum IgE concentrations were also elevated at 1,785 IU/ml (normal <310 IU/ml). Radioallergosorbent tests for multiple allergens were negative.Other normal laboratory values included antinuclear antibody, anti-DNA antibody, and complement titer.

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Interferon-α in the idiopathic hypereosinophilic syndrome: consideration of five cases

Cited by 60 publications

References 10 publications

The renaissance of interferon therapy for the treatment of myeloid malignancies

The renaissance of interferon therapy for the treatment of myeloid malignancies

The FIP1L1-PDGFRα fusion tyrosine kinase in hypereosinophilic syndrome and chronic eosinophilic leukemia: implications for diagnosis, classification, and management

A Rare Case of Idiopathic Hypereosinophilic Syndrome Involving the Oral Cavity Associated with the Esophagus and Gastrointestinal Tract

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