The Japanese Journal of Pediatric Hematology
Online ISSN : 1884-4723
Print ISSN : 0913-8706
ISSN-L : 0913-8706
Transient Abnormal Myelopoiesis with Trisomy 21 Karyotype on Leukemoid Blast Cells and Cutaneous Involvement
Nakao KONISHIHiroshi KASHIWAMasao KOBAYASHIYoshinori KAWAGUCHIYoshito TANAKAKazuhiro UEDA
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Keywords: TAM
JOURNAL FREE ACCESS

1989 Volume 3 Issue 4 Pages 367-371

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Abstract

Transient abnormal myelopoiesis (TAM) has been well described in infants with trisomy 21. We report a phenotypically normal newborn infant with hepatosplenomegaly, skin rash, and circulating blastoid cells. Chromosome analysis on peripheral blood cells incubated without PHA revealed 47XY, +21 on all cells.In contrast, peripheral blood cells stimulated with PHA for 72 hours had exclusively 46XY. A biopsy of the skin lesion exhibited infiltration of leukemoid blast cells. Without any anticancer drugs, the skin lesions as well as organomegaly gradually resolved, and leukemoid blasts disappeared from both the peripheral blood and the bone marrow. Subsequent chromosome analysis on peripheral blood cells disclosed normal karyotype. These findings suggest that an abnormal clone with 21 trisomy might be associated with the proliferation of leukemoid blasts in this patient with TAM.

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