Genetic Alterations Precede DNA Methylation Changes in Juvenile Myelomonocytic Leukemia

Abnormal promoter DNA methylation in juvenile myelomonocytic leukemia is not caused by mutation in DNMT3A.

Juvenile myelomonocytic leukemia.

A was less than 4 years, with any type of myelodysplasia and monosomy 7. All other patients, including older children BOUT 40 YEARS ago two forms of chronic myelocytic leukemia (CML) were recognized in children. One had the typical features of CML of adulthood and usually with monosomy 7, were classified according to FAB criteria. The revised classification led the authors to propose a new appe...

Aberrant DNA Methylation Is Associated with a Poor Outcome in Juvenile Myelomonocytic Leukemia

Juvenile myelomonocytic leukemia (JMML), an overlap of myelodysplastic / myeloproliferative neoplasm, is an intractable pediatric myeloid neoplasm. Epigenetic regulation of transcription, particularly by CpG methylation, plays an important role in tumor progression, mainly by repressing tumor-suppressor genes. To clarify the clinical importance of aberrant DNA methylation, we studied the hyperm...

PEDIATRIC HEMATOLOGY Juvenile myelomonocytic leukemia

Juvenile myelomonocytic leukemia (JMML) is a rare clonal myeloproliferative disorder (MPD) of early childhood [1]. The median age at diagnosis is 2 years [1]. There is a male predominance with a male:female ratio of 2:1. Pallor, fever, infection, skin bleeding and cough are the most common presenting symptoms. Typically, there is marked hepatosplenomegaly. JMML rarely involves the central nervo...

Pathogenic Mechanisms in Juvenile Myelomonocytic Leukemia

Juvenile myelomonocytic leukemia (JMML) is a relentless myeloproliferative disorder (MPD) of young children characterized by over-production of myeloid lineage cells that infiltrate hematopoietic and non-hematopoietic tissues including skin, lung, and spleen. The median survival is < 1 year without hematopoietic stem cell transplantation (HSCT). A cellular hallmark of JMML is that primary blood...