Myeloid Leukemia in Down Syndrome

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Myeloid leukemia associated with Down syndrome.

A 2-year-old girl with Down syndrome (DS) presented to the emergency department after developing easy bruising and a petechial rash on her arms, legs, and face. Her laboratory results on admission were significant for anemia and thrombocytopenia: white blood cells, 10.1 3 10/L; hemoglobin, 6.6 g/dL; hematocrit, 19.4%; mean corpuscular volume, 102.6 fL; red cell distribution width, 15.7%; and pl...

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Clonal selection in xenografted TAM recapitulates the evolutionary process of myeloid leukemia in Down syndrome.

Transient abnormal myelopoiesis (TAM) is a clonal preleukemic disorder that progresses to myeloid leukemia of Down syndrome (ML-DS) through the accumulation of genetic alterations. To investigate the mechanism of leukemogenesis in this disorder, a xenograft model of TAM was established using NOD/Shi-scid, interleukin (IL)-2Rγ(null) mice. Serial engraftment after transplantation of cells from a ...

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Myeloid Cell Leukemia-1 Gene Expression and Clinicopathological Features in Myelodysplastic Syndrome

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Clinical characteristics and outcome of refractory/relapsed myeloid leukemia in children with Down syndrome.

Myeloid leukemia in Down syndrome (ML-DS) is associated with good response to chemotherapy and favorable prognosis. Because little research has been focused on refractory/relapsed (R/R) cases, we conducted a retrospective analysis for R/R ML-DS. Among ML-DS patients diagnosed between 2000 and 2010 in Japan, 26 relapsed (25 in the BM and 1 in the skin), and 3 refractory patients were enrolled. T...

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BACKGROUND Children with Down syndrome (DS) have increased risk for developing AML (DS-AMKL), and they usually experience severe therapy-related toxicities compared to non DS-AMKL. Refractory/relapsed disease has very poor outcome, and patients would benefit from novel, less toxic, therapeutic strategies that overcome resistance. Relapse/resistance are linked to cancer stem cells with high alde...

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ژورنال

عنوان ژورنال: Critical Reviews™ in Oncogenesis

سال: 2011

ISSN: 0893-9675

DOI: 10.1615/critrevoncog.v16.i1-2.40