Durable remission after treatment with very low doses of imatinib for FIP1L1-PDGFRα-positive chronic eosinophilic leukaemia

Helbig, Grzegorz; Moskwa, Andrzej; Hus, Marek; Piszcz, Jarosław; Świderska, Alina; Urbanowicz, Alina; Całbecka, Małgorzata; Seferyńska, Ilona; Raźny, Małgorzata; Rodzaj, Marek; Zuk, Ewa; Kyrcz‐Krzemień, Sławomira

doi:10.1007/s00280-011-1582-3

Cited by 23 publications

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“…We found that patients with CEL-NOS were predominantly male (70%), and splenomegaly (70%) was the most frequent clinical manifestation. These findings were consistent with the results reported for F/P-positive CEL patients [8,9]. We attempted to compare the results of blood tests, which have been reported for F/P-positive CEL patients by two large European groups [8,10] with those provided in this study.…”

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confidence: 89%

Chronic eosinophilic leukemia‐not otherwise specified has a poor prognosis with unresponsiveness to conventional treatment and high risk of acute transformation

et al. 2012

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Chronic eosinophilic leukemia-not otherwise specified (CEL-NOS) is a rare disorder with hypereosinophilia and an increased number of blood or marrow blast (<20%) or an evidence of eosinophil clonality. We evaluated the clinical outcome of 10 patients with CEL-NOS. Seven males and three females at a median age of 62 years (range, 23-73) were included. The median leukocyte count at diagnosis was 33.4 3 10 9 /l (range, 9.3-175.0) with a median eosinophil count of 15.6 3 10 9 /l (range, 1.5-136.0). Median hemoglobin and platelets were 11.0 g/dl (range, 8.3-13.3) and 158 3 10 9 /l (range, 31.0-891.0), respectively. Clinical manifestations included splenomegaly (n 5 7), hepatomegaly (n 5 6), cardiac failure (n 5 2), and lung infiltrations (n 5 1). Median survival from diagnosis to death for entire cohort was 22.2 months (range, 2.2-186.2). Five of the 10 studied patients developed acute transformation (AT) after median of 20 months from diagnosis (range, 1.6-41.9). None of patients with AT is alive at the time of last follow-up. Median time from AT to death was 2 months (range, 1.0-6.1). Among five patients who did not develop AT, three died in active disease. Two patients are alive in complete remission; first underwent allogeneic stem-cell transplantation preceding by intensive induction chemotherapy; the second remains on imatinib with hydroxyurea. Except the latter patient, imatinib was ineffective in our study population. CEL-NOS is a rare and aggressive disease with high rate of AT and resistance to conventional treatment.Chronic eosinophilic leukemia-not otherwise specified (CEL-NOS) is a rare Philadelphia-negative myeloproliferative neoplasm, which is due to clonal proliferation of eosinophil precursors. Peripheral blood hypereosinophilia (>1.5 3 10 9 /l), an increased number of myeloblasts in blood or marrow (<20%) or an evidence of eosinophil clonality, are required for diagnosis. The cases of myeloid and lymphoid neoplasms with abnormalities of platelet-derived growth factor alpha (PDGFRA), platelet-derived growth factor beta (PDGFRB), and fibroblast growth factor receptor 1 (FGFR1) must be also excluded [1]. In a new WHO classification, CEL-NOS is listed in the category of myeloproliferative neoplasms [2], whereas it was incorporated under the heading of myeloproliferative forms of hypereosinophilic syndromes according to the classification of the Eosinophilic Working Group [3]. Regardless of the classification used, CEL-NOS remains an extremely rare entity with variable prognosis [1,4]. The efficacy of currently used therapeutic agents is limited, and the hematological responses are usually short-lived [5].Ten patients (seven males and three females) at a median age of 62 years (range, 23-73) were included in this study. The median leukocyte count at diagnosis was 33.4 3 10 9 /l with a median eosinophil count of 15.6 3 10 9 /l. Blood smear was dominated by mature eosinophils with a small number of younger forms, for example, eosinophilic myelocytes and promyelocytes. Single eosinophils had dysplastic fe...

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Chronic eosinophilic leukemia‐not otherwise specified has a poor prognosis with unresponsiveness to conventional treatment and high risk of acute transformation

et al. 2012

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“…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. Esto traduce una mayor sensibilidad general que LMC al tratamiento con ITK de primera línea 9,13 . En relación a la duración del tratamiento, se recomienda que éste sea permanente y en la dosis a la cual se haya producido respuesta.…”

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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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“…This patient achieved long-term remission following the administration of intensive chemotherapy and 100 mg daily IM maintenance therapy. It has been shown in the literature that treatment with low-dose IM provides the same therapeutic benefits for patients with FIP1L1-PDGFRA -associated MPN [23,24], but the optimal therapeutic dose for the patients with both FIP1L1-PDGFRA -associated MPN and MS remains uncertain. The patient in the current report had not progressed to AML.…”

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confidence: 99%

Complete Response of Myeloid Sarcoma with <b><i>FIP1L1-PDGFRA</i></b>-Associated Myeloproliferative Neoplasms to Imatinib Mesylate Monotherapy

2012

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Myeloid sarcoma (MS) is a localized, extramedullary tumor of acute myeloid leukemia (AML) that typically presents either de novo or concomitantly with myeloproliferative neoplasms (MPN), AML and myelodysplastic syndrome. Patients who have MS must be treated with intensive chemotherapy, as are patients with AML, because MS usually progresses to a systemic manifestation and leads to dismal outcomes. FIP1L1-PDGFRA-associated MPN, a subtype of myeloid and lymphoid neoplasm, is characterized by eosinophilia and abnormalities in the PDGFRA, PDGFRB or FGFR1 gene. Fusion of the FIP1L1 and PDGFRA genes activates the tyrosine kinase. As a result, imatinib mesylate (IM) is widely used for the treatment of this disorder. The coexistence of FIP1L1-PDGFRA-associated MPN and MS is extremely rare. Patients with this condition fail to achieve durable remission and long-term survival without a combination of intensive chemotherapy and IM. Here, we report a case of MS and FIP1L1-PDGFRA-associated MPN that was successfully treated with IM monotherapy.

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Durable remission after treatment with very low doses of imatinib for FIP1L1-PDGFRα-positive chronic eosinophilic leukaemia

Cited by 23 publications

References 12 publications

Chronic eosinophilic leukemia‐not otherwise specified has a poor prognosis with unresponsiveness to conventional treatment and high risk of acute transformation

Chronic eosinophilic leukemia‐not otherwise specified has a poor prognosis with unresponsiveness to conventional treatment and high risk of acute transformation

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

Complete Response of Myeloid Sarcoma with <b><i>FIP1L1-PDGFRA</i></b>-Associated Myeloproliferative Neoplasms to Imatinib Mesylate Monotherapy

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