Abstract

Klinefelter syndrome (KS) is the most frequent type of congenital sex-chromosomal disorder caused by at least one extra X chromosome and commonly treated with lifetime testosterone therapy. Ulcerative lesions on lower extremities may occur as a complication of KS. The pathogenesis of ulcers in KS patients has not been clarified on a molecular level. Here we present a case of leg ulcers exacerbation associated with the administration of a high dose of acetylsalicylic acid in a 63-year-old KS patient with karyotype 47,XXY undergoing testosterone replacement therapy for the last 20 years. The appearance of the ulcer on the patient's leg occurred during one week of high oral acetylsalicylic acid intake (1.2 g daily). The patient was advised to return to his standard daily dose of 0.1 g of acetylsalicylic acid and significant improvement of his leg ulcer was observed after two weeks. We hypothesize that testosterone-mediated nitric oxide balance in KS patient is perturbed under the condition of acute high-dose acetylsalicylic acid administration. We propose that small standard doses of approximately 0.1 g/day of acetylsalicylic acid have no apparent effect on nitric oxide status, whereas higher doses may cause dysregulation of nitric oxide production and/or utilization, creating conditions which may cause the appearance of leg ulcers in the KS patients.

Highlights

  • Klinefelter syndrome (KS) is the most frequent type of congenital sex-chromosomal disorder affecting approximately 1:660 newborn males, and it is a rather common cause of infertility, hypogonadism and learning disability [1]

  • We present a case of leg ulcers exacerbation associated with the administration of a high dose of acetylsalicylic acid in a 63-year-old KS patient with karyotype 47,XXY undergoing testosterone replacement therapy for the last 20 years

  • The patient was advised to return to his standard daily dose of 0.1 g of acetylsalicylic acid and significant improvement of his leg ulcer was observed after two weeks

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Summary

Introduction

Klinefelter syndrome (KS) is the most frequent type of congenital sex-chromosomal disorder affecting approximately 1:660 newborn males, and it is a rather common cause of infertility, hypogonadism and learning disability [1]. We hereby report the case of a 63-year-old KS patient with 47,XXY genotype on a supplemental testosterone therapy who took 1.2 g of acetylsalicylic acid per day for a week. During this period of high-dose acetylsalicylic acid intake, the ulcer on the left leg of the KS patient appeared and gradually worsened. The patient has experienced several episodes of leg ulceration often associated with an open bleeding wound that occurred simultaneously with the consumption of a high dose of acetylsalicylic acid. We concluded that the blood analysis of biochemical and immunological parameters suggests that the patient's leg ulcer was not related to deep vein thrombosis and metabolic syndrome

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