9946/3 Juvenile myelomonocytic leukemia


Synonyms

Juvenile chronic myelomonocytic leukemia

Definitions

Tumours of haematopoietic and lymphoid tissues
ICD-O-3 topography code: C42 and C77
ICD10: C93.3
           

/span>uvenile myelomonocytic leukaemia (JMML) is a clonal haematopoietic disorder of childhood characterized by proliferation principally of the granulocytic and monocytic lineages. Blasts plus promonocytes account for <20% of cells in peripheral blood (PB) and bone marrow (BM). Erythroid and megakaryocytic abnormalities are frequently present 1
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Amenomori T, Tomonaga M, Yoshida Y, Kuriyama K, Matsuo T, Jinnai I, Ichimaru M, Omiya A, Tsuji Y (1986)
Cytogenetic evidence for partially committed myeloid progenitor cell origin of chronic myelomonocytic leukaemia and juvenile chronic myeloid leukaemia: both granulocyte-macrophage precursors and erythroid precursors carry identical marker chromosome.
Br J Haematol 64: 539-46



2
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Busque L, Gilliland DG, Prchal JT, Sieff CA, Weinstein HJ, Sokol JM, Belickova M, Wayne AS, Zuckerman KS, Sokol L (1995)
Clonality in juvenile chronic myelogenous leukemia.
Blood 85: 21-30



3
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Miles DK, Freedman MH, Stephens K, Pallavicini M, Sievers EL, Weaver M, Grunberger T, Thompson P, Shannon KM (1996)
Patterns of hematopoietic lineage involvement in children with neurofibromatosis type 1 and malignant myeloid disorders.
Blood 88: 4314-20



. BCR-ABL1 is absent, whereas mutations involving genes of the RAS/MAPK pathway are characteristic.

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