1998
DOI: 10.1007/s002770050411
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Transient myeloproliferative disorder with 11q23 aberration in two neonates with Down syndrome

Abstract: Infants with Down syndrome may develop a transient myeloproliferative disorder (TMD) with the features of acute leukemia but resolving in a spontaneous remission. Chromosomal aberrations in addition to trisomy 21 have only rarely been described. In many cases of infant acute leukemia band q23 of chromosome 11 is involved in nonrandom translocations, often resulting in a rearrangement of the ALL-1 (MLL, HRX, HTRX 1) gene. Generally, this translocation carries a bad prognosis. We describe two newborn girls with … Show more

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Cited by 9 publications
(3 citation statements)
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“…The marrow milieu or target cell may have been unfavorable. The KMT2A‐ R clone may have been obliterated either immunologically 43–49 or by ensuing myeloablative treatment; however, therapy‐related KMT2A ‐R leukemia has occurred on MSK‐N7 5 . The latency may prove even longer.…”
Section: Resultsmentioning
confidence: 99%
“…The marrow milieu or target cell may have been unfavorable. The KMT2A‐ R clone may have been obliterated either immunologically 43–49 or by ensuing myeloablative treatment; however, therapy‐related KMT2A ‐R leukemia has occurred on MSK‐N7 5 . The latency may prove even longer.…”
Section: Resultsmentioning
confidence: 99%
“…Additional chromosomal abnormalities are common in DS myeloblasts of patients with AML and occasionally in TMD [16,17]. There have been a variety of opinions regarding the nature of TMD.…”
Section: R Ole Of C H R Om Osom E 21 In Leuk Em Ogenesismentioning
confidence: 99%
“…2 However, there may be diagnostic difficulty in some cases as there have been reports of TMD at later ages (second or third month of life), as well as cases of "congenital leukemia". 4 Hematologic and cytogenetic differences between these disorders also have been described. TMD tends to manifest with normal hematocrit and platelet counts, whereas AML generally exhibits cytopenias.…”
Section: Introductionmentioning
confidence: 99%