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  • Le Syndrome de Klinefelter
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Testicule et sperme dans le Syndrome de Klinefelter chez l'adulte

Testis and semen in Klinefelter's syndrome in adult

Andrologie volume 4, pages 296–302 (1994)Cite this article

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Dans sa forme typique le syndrome de Klinefelter comporte l'azoospermie, l'hypogonadisme hypogonadotrope et souvent une gynécomastie. L'examen microscopique du testicule montre une fibro-hyalinose avec épaississement de la gaine péri-tubulaire et réduction du diamètre des tubes séminifères.

L'épithélium séminal est constitué par les cellules de Sertoli et inconstamment par des germinales immatures. On observe exceptionnellement des spermatozoïdes. La glande interstitielle parait hyperplasique, parfois pseudo-adénomateuse. Elle sécrète peu d'hormones stéroïdes.

L'azoospermie est un des signes majeurs du syndrome de Klinefelter mais elle n'est pas constante, parfois limitée à une oligozoospermie sévère. Dans ce cas une certaine fécondité peut être conservée, souvent associée à une constitution chromosomique en mosaïque. Les lésions testiculaires tendent à s'aggraver avec l'âge.

Abstract

Klinefelter's syndrome is characterised by azoospermia, gynecomastia and hypogonadotropic hypogonadism. The testes are reduced in volume. Microscopically, seminiferous tubules show a reduced diameter and a thickening of peritubular sheath. The seminiferous epithelium contains Sertoli cells with a clear typical nucleus and a heterogeneous nucleolus. Usually there are no germ cells. When present they degenerate at the spermatocyte stage. Very few achieve a complete maturation until the spermatozoon form.

The interstitial gland appears relatively hypertrophied, due to the decrease in volume in the other components of the testis. Sometimes Leydig cells display an adenomatous morphology. Therefore the steroidogenic function is impaired.

Azoospermia is a major event in Klinefelter's syndrome but sometimes restricted to severe oligozoospermia. In that cases, fertility may be somewhat preserved, and the caryotype usually reveals a mosaicism, 47,XY/47,XXY with a better prognosis than in pure syndrome.

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  1. Laboratoire de Biologie de la Fertilité et CECOS, Hôtel-Dieu de Paris, Place du Parvis Notre-Dame, Paris

    Eric Vendrely

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Vendrely, E. Testicule et sperme dans le Syndrome de Klinefelter chez l'adulte. Androl. 4, 296–302 (1994). https://doi.org/10.1007/BF03034318

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Basic and Clinical Andrology

ISSN: 2051-4190