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Transient myeloproliferative disorder in chromosomally normal newborn infant

✍ Scribed by Kalousek, D. K. ;Chan, K. W.

Publisher
John Wiley and Sons
Year
1987
Tongue
English
Weight
349 KB
Volume
15
Category
Article
ISSN
0098-1532

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✦ Synopsis

Transient myeloproliferative disorder is a condition clinically resembling congenital acute myelogenous leukemia. As in acute leukemias the blast cell population in this disorder may have either normal or abnormal chromosomal complement. Transient myeloproliferative disorder is well recognized in neonates with a complete or mosaic trisomy 21 (Down's syndrome). We report a phenotyp ically and cytogenetically normal infant with this syndrome in whom only blast cells showed trisomy 21. We postulate that the pathogenesis of the transient rnyeloproliferative disorder in both Down's syndrome infants and those with a normal chromosomal complement is related to abnormal prenatal production of placental regulatory hemopoietic factors caused by chromosomal defect(s) confined to placental tissues.

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Immunophenotype of a transient myeloprol
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We report the prenatal diagnosis of a transient myeloproliferative disorder suggestive of leukaemia in a fetus with hepatosplenomegaly, hydrops and 47, XY,+21 karyotype. The initial fetal white blood cell count at 26+5 weeks' gestation was 190/nl with 70 per cent blast cells. Immunophenotyping of th

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## Abstract Transient myeloproliferative disorder is a unique disease in infants with Down syndrome that typically resolves spontaneously. In this report, we present a fatal case of transient myeloproliferative disorder with unusual sonographic findings including adrenal gland enlargement and thick