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Chimerism in twins: Caution is needed in interpretation of karyotypes

✍ Scribed by Sylvie Bourthoumieu; Francoise Esclaire; Catherine Yardin


Publisher
John Wiley and Sons
Year
2006
Tongue
English
Weight
64 KB
Volume
140A
Category
Article
ISSN
1552-4825

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✦ Synopsis


Interpretation of cytogenetic analysis in twins needs particular care and attention, as shown by the case of Johannsen et al. [2003], in which a child's ovaries were unnecessarily removed based on misleading cytogenetic information. Briefly, the authors reported a case of suspected monozygotic (MZ) twins with discrepant sex phenotype at birth, whereas the amniocentesis and the ultrasound examination had pointed towards male sex of both twins. In fact, at birth one twin presented as a normal boy, and the other twin as a phenotypically normal female with 46,XY blood karyotype. Gonadal dysgenesis was diagnosed, and gonadectomy was performed in the phenotypically female twin to prevent the development of a gonadoblastoma. However, the histology of the gonads revealed normal infantile ovaries, and chromosomal analysis of fibroblasts from a skin biopsy demonstrated a 46,XX karyotype. This newborn was in fact a 46,XX female with a chimeric 46,XY blood cell line due to intrauterine transfusion from her twin brother.