1990
DOI: 10.1002/mpo.2950180502
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Acute nonlymphocytic leukemia after transient myeloproliferative disorder in a patient with down syndrome

Abstract: An infant with Down syndrome (DS) and RH isoimmunization developed transient myeloproliferative disorder (TMD) during the neonatal period. At 16 months she presented with acute nonlymphocytic leukemia (ANLL). Cytogenetic studies during TMD showed trisomy 21 only but new abnormalities emerged during ANLL. She is now in complete remission 5 years after diagnosis. Patients with TMD have either trisomy 21 or mosaic 21 in blood and bone marrow but in phenotypically normal children this cell line disappears with res… Show more

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Cited by 19 publications
(11 citation statements)
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References 34 publications
(29 reference statements)
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“…An acquired chromosome 21 (i.e. tetrasomy 21) t(21;21) and i21 are most common ( Barnett et al , 1990 ).…”
Section: Transient Myeloproliferative Disordermentioning
confidence: 99%
See 1 more Smart Citation
“…An acquired chromosome 21 (i.e. tetrasomy 21) t(21;21) and i21 are most common ( Barnett et al , 1990 ).…”
Section: Transient Myeloproliferative Disordermentioning
confidence: 99%
“…If leukaemia develops following TMD, it is almost always AMKL and it usually occurs between the ages of 1 and 3 years ( Lin et al , 1980 ; Nito et al , 1983 ; Ruiz‐Argielles et al , 1986 ; Barnett et al , 1990 ; Zipursky et al , 1992 ). That both TMD and AML are usually phenotypically AMKL suggests that the second disease is derived from the first.…”
Section: Transient Myeloproliferative Disordermentioning
confidence: 99%
“…Unlike TL, if left untreated, the subsequent leukemia does not spontaneously regress. 4,5,15,[17][18][19] In 1996, the Pediatric Oncology Group (POG) initiated the first prospective study (POG 9481) to look at the clinical features, biology, and natural history of TL in children with DS (or trisomy 21 mosaicism), and to identify DS infants at risk for early death or development of subsequent leukemia.…”
Section: Introductionmentioning
confidence: 99%
“…Most of the information about the natural history and biology of this TL has been gleaned from sporadic case reports and/or retrospective reviews of medical and/or pathologic records 4,5,15,17. Although the disease may spontaneously disappear, a significant percentage of patients with TL go on to develop AMKL within the first 4 years of life, which if left untreated rarely shows spontaneous regression a second time 4,5,15,17–19. The Pediatric Oncology Group Study 9481 was the first study designed to prospectively study the clinical presentation and natural history of DS children (or trisomy 21 mosaics) with TL, to determine the immunophenotypic, cytogenetic, and clonal characteristics of the blast cells in this disorder, and to assess their prognostic significance for the development of acute non‐lymphocytic leukemia.…”
Section: Clinical Characteristics At Presentation Of Patients With Trmentioning
confidence: 99%