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1987•Journal of Medical GeneticsOpen access

Unilateral true hermaphrodite with 46,XX/46,XY dispermic chimerism.

Talaat I. Farag, S A Al-Awadi, Patricia Tippett, Mohammed Abd Elaziz ElSayed, T.S. Sundareshan, S. A. Al‐Othman, Magda H. El‐Badramany

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Abstract

A 13 year old female presented with ambiguous external genitalia, right inguinal ovotestis, left ovary, apparently normal Mullerian system, and absent Wolffian system. Cultured lymphocytes showed a 46,XX/46,XY karyotype. Histopathology of the gonads confirmed true hermaphroditism. The presence of two genetically different erythrocyte populations was observed. The findings suggested that the patient is a true hermaphrodite dispermic chimera.

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A 13 year old female presented with ambiguous external genitalia, right inguinal ovotestis, left ovary, apparently normal Mullerian system, and absent Wolffian system. Cultured lymphocytes showed a 46,XX/46,XY karyotype. Histopathology of the gonads confirmed true hermaphroditism. The presence of two genetically different erythrocyte populations was observed. The findings suggested that the patient is a true hermaphrodite dispermic chimera.

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Available abstract

A 13 year old female presented with ambiguous external genitalia, right inguinal ovotestis, left ovary, apparently normal Mullerian system, and absent Wolffian system. Cultured lymphocytes showed a 46,XX/46,XY karyotype. Histopathology of the gonads confirmed true hermaphroditism. The presence of two genetically different erythrocyte populations was observed. The findings suggested that the patient is a true hermaphrodite dispermic chimera.

Key concepts: Ovotestis, True hermaphroditism, Hermaphrodite, Biology, Karyotype, Disorders of sex development, Chimera (genetics), Gonad

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