The efficacy of imatinib mesylate in patients with FIP1L1-PDGFR -positive hypereosinophilic syndrome. Results of a multicenter prospective study

Baccarani, Michele; Cilloni, Daniela; Rondoni, Michela; Ottaviani, Emanuela; Messa, Francesca; Merante, S.; Tiribelli, Mario; Buccisano, Francesco; Testoni, Nicoletta; Gottardi, Enrico; Vivo, Antonio De; Gatto, Emilia; Iacobucci, Ilaria; Paolini, Stefania; Soverini, Simona; Rosti, Gianantonio; Rancati, Francesca; Astolfi, C.; Pane, Fabrizio; Saglio, Giuseppe

doi:10.3324/haematol.11420

Cited by 175 publications

(184 citation statements)

References 41 publications

(42 reference statements)

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“…16 Unfortunately, many patients show only a transient or partial response and require treatment with additional agents. Although reported imatinib response rates in PDGFRA-negative HES vary widely (9-60%) depending on the series, 16,[78][79][80] recent data from our center suggest that the presence of myeloproliferative features (presumed clonal eosinophilic involvement) is an important predictor of imatinib response in patients with FIP1L1-PDGFRA-negative HES. Of note, PDGFR-negative patients often require higher doses of imatinib and appear to respond more slowly.…”

Section: Pdgfr-negative M-hesmentioning

confidence: 99%

How I treat hypereosinophilic syndromes

Klion

2015

Blood

193

126

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Hypereosinophilic syndromes (HESs) are a group of rare disorders characterized by peripheral blood eosinophilia of 1.5 × 109/L or higher and evidence of end organ manifestations attributable to the eosinophilia and not otherwise explained in the clinical setting. HESs are pleomorphic in clinical presentation and can be idiopathic or associated with a variety of underlying conditions, including allergic, rheumatologic, infectious, and neoplastic disorders. Moreover, the etiology of the eosinophilia in HESs can be primary (myeloid), secondary (lymphocyte-driven), or unknown. Although corticosteroids remain the first-line therapy for most forms of HESs, the availability of an increasing number of novel therapeutic agents, including tyrosine kinase inhibitors and monoclonal antibodies, has necessarily altered the approach to treatment of HESs. This review presents an updated treatment-based approach to the classification of patients with presumed HES and discusses the roles of conventional and novel agents in the management of these patients.

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Section: Pdgfr-negative M-hesmentioning

confidence: 99%

How I treat hypereosinophilic syndromes

Klion

2015

Blood

193

126

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“…Al documentarse la actividad inhibitoria de imatinib sobre otros tipos de receptores tirosina quinasa desregulados (PDGFRA, PDGFRB y c-kit), se iniciaron test intentado probar su utilidad en otras neoplasias, como la CEL, obteniendo respuestas duraderas con menores dosis del fármaco 2,11 . En este sentido; un estudio italiano prospectivo demostró, en una serie de 27 pacientes con documentación de la presencia del gen de fusión FIP1L1/PDGFRA y dosis de imatinib 100-400 mg/día, remisión hematológica completa y sostenida en un seguimiento de 25 meses 12 . Asimismo, en otra serie de 40 pacientes, se reportó remisión hematológica por más de un año, aún en aquéllos tratados con 100 mg/día de imatinib.…”

Section: Discussionunclassified

Leucemia eosinofílica crónica con respuesta hematológica sostenida tras tratamiento con bajas dosis de imatinib

Chandía

2014

Rev. méd. Chile

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Fax: 56-71-413657 demetriotorresc@gmail.com L a eosinofilia en sangre o tejidos puede ser producida por una gran variedad de situaciones clínicas: desde cuadros autolimitados hasta enfermedades de riesgo vital. La gravedad de las manifestaciones clínicas está directamente relacionada con el tiempo de exposición y magnitud del recuento de eosinófilos. El término hipereosinofilia se refiere a recuentos sostenidos de eosinófilos superiores a 1.500/mL en sangre periférica, los que pueden producir daño e infiltración en órganos diana independiente de la causa que lo provoque 1 .El término leucemia eosinofílica crónica (CEL) alude a un subgrupo de neoplasias mieloproliferativas, caracterizada por hipereosinofilia en médula ósea y sangre periférica; asociada a variable compromiso de mastocitos y neutrófilos. En su forma pura, es secundaria a alteraciones cromosómicas.De ellas, la más frecuente, es la ocasionada por el reordenamiento 4q12 que origina el producto de fusión FIP1L1/PDGFRA. Otras causas menos frecuentes son los reordenamientos en los genes PDGFRB y FGFR1, asociándose también este último a neoplasias linfoides 2-4 . A la fecha no existen reportes de su prevalencia en Chile y el subdiagnóstico dificulta la estimación de su real presencia en la población.Se presenta el caso de un paciente con CEL asociada a reordenamiento de PDGFRA, con rápi-da y sostenida respuesta a inhibidores de tirosina quinasa (ITK). Caso clínicoPaciente 58 años de edad, sexo masculino, previamente sano, sin antecedente de uso de medica-

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“…There are also reports about the use of cromolyn, intravenous gamma globulin, vincristine, cyclosporin A, tyrosine kinase inhibitor imatinib mesylate, and antiinterleukin-5 agent mepolizumab 5,[31][32][33] . In the presence of either PDGFRA or PDGFRB mutations, the use of imatinib has been shown effective 11,34 . Routine cardiac therapy with digitalis, diuretics, afterload reduction, and anticoagulation are adjuncts in the management of these patients and should be initiated early 6 .…”

Section: Discussionmentioning

confidence: 99%

Large Apical Thrombus in a Patient with Persistent Heart Failure and Hypereosinophilia: Löffler Endocarditis

et al. 2008

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Idiopathic hypereosinophilic syndrome is an uncommon leukoproliferative systemic disorder characterized by the overproduction of eosinophils and poor prognosis. A major source of morbidity and mortality of this syndrome is the associated cardiac involvement represented by endocardial thickening and mural thrombi. We report a 64-year-old woman with persistent symptoms of heart failure despite standard medical therapy. Echocardiography revealed reduced left ventricular filling due to a large apical mass; an abnormal diastolic filling pattern was also noticed. Complete blood count revealed remarkable hypereosinophilia. Cardiac magnetic resonance imaging demonstrated an apical thrombus and intense linear enhancement of the endocardium, which were compatible with Löffler endocarditis. Medical therapy, including corticosteroids and anticoagulation, was initiated promptly. The symptoms improved as the peripheral hypereosinophilia resolved in 15 days. The patient was asymptomatic at the 1-year follow-up visit with complete regression of the apical thrombus and no evidence of restrictive cardiomyopathy. We report this case to draw attention to this particularly rare condition with poor prognosis since quick and accurate diagnosis and prompt initiation of therapy may improve symptoms and survival.

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The efficacy of imatinib mesylate in patients with FIP1L1-PDGFR -positive hypereosinophilic syndrome. Results of a multicenter prospective study

Cited by 175 publications

References 41 publications

How I treat hypereosinophilic syndromes

How I treat hypereosinophilic syndromes

Leucemia eosinofílica crónica con respuesta hematológica sostenida tras tratamiento con bajas dosis de imatinib

Large Apical Thrombus in a Patient with Persistent Heart Failure and Hypereosinophilia: Löffler Endocarditis

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