Mutations in the let-7 binding site - a mechanism of RAS activation in juvenile myelomonocytic leukemia?
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چکیده
منابع مشابه
Mutations in the let-7 binding site - a mechanism of RAS activation in juvenile myelomonocytic leukemia?
Juvenile myelomonocytic leukemia (JMML) is a rare hematologic malignancy in childhood and accounts for less than 3% of all childhood hematologic malignancies. The role of hyperactive RAS in JMML is underlined by the fact that approximately 80% of JMML develop due to gain-of-function mutations in NRAS, KRAS, PTPN11 and SOS1 or homozygous loss-of-function mutations in NF1 or c-CBL. These genes ar...
متن کاملSpontaneous improvement of hematologic abnormalities in patients having juvenile myelomonocytic leukemia with specific RAS mutations.
Of 11 children with juvenile myelomonocytic leukemia (JMML) carrying RAS mutations (8 with NRAS mutations, 3 with KRAS2 mutations), 5 had a profound elevation in either or both the white blood cells and spleen size at diagnosis. Three patients had no or modest hepatosplenomegaly and mild leukocytosis at presentation but subsequently showed a marked increase in spleen size with or without hemato...
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In a recent issue of Blood, Matsuda et al reported 11 children with juvenile myelomonocytic leukemia (JMML) and clonal NRAS or KRAS mutations.1 Three patients showed improvement of various clinical and laboratory features over a 2to 4-year period without chemotherapy or hematopoietic stem cell transplantation (HSCT). The authors correlate the comparatively mild course with a specific mutation p...
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Juvenile myelomonocytic leukemia is an aggressive myeloproliferative disorder characterized by malignant transformation in the hematopoietic stem cell compartment with proliferation of differentiated progeny. Seventy-five percent of patients harbor mutations in the NF1, NRAS, KRAS, or PTPN11 genes, which encode components of Ras signaling networks. Using single nucleotide polymorphism arrays, w...
متن کاملDetermination of Monosomy 7 among In Vitro Generated CD1a Positive Cells in Patients with Juvenile Myelomonocytic Leukemia
Objective: To study whether the generated CD1a positive cells belong to the leukemic cells among patients with juvenile myelomonocytic leukemia. Materials and Methods: We used mononuclear cells from 3 patients with juvenile myelomonocytic leukemia, from which two had monosomy 7. The mononuclear cells from these patients were cultured in RPMI/10%FCS without adding exogeneous growth factors fo...
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ژورنال
عنوان ژورنال: Haematologica
سال: 2010
ISSN: 0390-6078,1592-8721
DOI: 10.3324/haematol.2010.024984