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CASE REPORT

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TESTOSTERONE-INDUCED PRIAPISM INKLINEFELTER SYNDROME

KENTARO ICHIOKA, NORIAKI UTSUNOMIYA, NAOKI KOHEI, NOBUFUMI UEDA,KOJI INOUE, AND AKITO TERAI

ABSTRACTe present the case of a patient with Klinefelter syndrome and priapism after testosterone administration. The

atient underwent testicular sperm extraction for interventional fertilization, but no sperm were found. Becausee had given up trying to father a child and his testosterone level had been low, testosterone replacement therapyas started with testosterone enanthate. After the third injection, he presented with low-flow priapism. Conser-ative management was successful, and no recurrence developed during the next 15 months. Testosteroneeplacement therapy carries some risk of priapism even for patients with Klinefelter syndrome. UROLOGY 67:22.e17–622.e18, 2006. © 2006 Elsevier Inc.

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linefelter syndrome is the most common ge-netic cause of male infertility. Recent technical

evelopments in testicular sperm retrieval and intra-ytoplasmic sperm injection have made fathering ahild possible even for patients with Klinefelter syn-rome. However, in some cases, testicular spermannot be retrieved, and, if the serum testosteroneoncentrations are low, testosterone replacementherapy should be considered to avoid symptomsf androgen deficiency.We present a case of Klinefelter syndrome with

riapism after testosterone administration.

CASE REPORT

A 41-year-old man presented to our hospitalith infertility. The physical examination revealedynecomastia and bilateral testicular atrophy. Hiserum testosterone was 0.57 ng/mL (normal 2.5 to1.0), luteinizing hormone 29.5 mIU/mL (nor-al 2.0 to 12.0), and follicle-stimulating hormone

2.2 mIU/mL (normal 1.0 to 12.0). Prolactin (4.1IU/mL, normal 2.4 to 13.0) was within the normal

ange. Multiple semen analyses confirmed azoosper-ia. After receiving informed consent, a cytogenetic

nvestigation was performed on the chromosomes

rom the Department of Urology, Kurashiki Central Hospital,kayama, JapanAddress for correspondence: Kentaro Ichioka, M.D., Department

f Urology, Kyoto Katsura Hospital, 17, Yamadahirao-cho, Nish-kyo-ku, Kyoto 615-8256, Japan. E-mail: ichioka@mq.0038.net

Submitted: June 24, 2005, accepted (with revisions): September

n7, 2005

2006 ELSEVIER INC.LL RIGHTS RESERVED

btained from peripheral blood lymphocytes. Thearyogram revealed 46,XY/47,XXY/48,XXXY mosa-cism, indicating mosaic Klinefelter syndrome.

Testicular sperm retrieval was attempted, but noperm were found. Because the patient had given uprying to father a child and complained of decreasingibido and potency, testosterone replacement therapyas started. Treatment was instituted with testoster-ne enanthate 250 mg intramuscularly every 4eeks. Although the patient’s potency was very lowefore treatment, after the injections, his potency andexual activity recovered.The third injection was given in error 12 days

fter the second injection. Eight days after the thirdnjection, a morning erection without sexual stim-lation lasted for 6 hours, and the patient pre-ented with painful priapism. He had no history oferineal trauma, malignancy, sickle cell anemia, orse of other medications. Aspiration of the corpusavernosum revealed dark blood with a partial ox-gen pressure of 3 mm Hg and a carbon dioxideressure of 100 mm Hg, confirming low-flow pri-pism. After aspiration and injection of adrenaline,omplete detumescence was achieved. The patientesumed testosterone therapy with testosteronenanthate 250 mg every 4 weeks, without recur-ence of priapism during the next 15 months.

COMMENT

The mechanism by which testosterone adminis-ration causes priapism in patients with hypogo-

adism is unclear—several mechanisms are possi-

0090-4295/06/$32.00doi:10.1016/j.urology.2005.09.041 622.e17

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le. In our case, the interval between the secondnd third injection was short. Accumulation of tes-osterone enanthate and a supraphysiologic localestosterone level could explain the priapism inur case. Zargooshi1 reported a case of priapism afterigh-dose testosterone therapy in a patient with hy-ogonadotropic hypogonadism. He concluded thatestosterone-induced priapism was dose dependent.n our case, priapism occurred 8 days after the thirdnjection. However, the serum concentration of tes-osterone is usually not that great 8 days after an in-ection, because the terminal half-life of testosteronenanthate is 4.5 days. However, the serum concentra-ion of testosterone was not measured in our patient.

Sudden exposure to testosterone after a long periodf deprivation may be a cause of priapism. Our pa-ient had been exposed to an extremely low testoster-ne level for a long period, and the local sensitivity toestosterone may have been elevated. In these pa-ients, the threshold for arousal may be lower thann patients with slightly decreased testosterone lev-ls. Shergill et al.2 reported a case of testosterone-

nduced priapism in a patient with Kallmann syn- s

22.e18

rome. Whalen et al.3 reported 2 cases of priapismn patients with hypogonadotropic hypogonadismho were receiving gonadotropin-releasing hor-one. These cases support the hypothesis of in-

reased local sensitivity to testosterone.When testosterone serum concentrations in

atients with Klinefelter syndrome are low, life-ong substitution therapy is indicated, if preser-ation of fertility is not necessary.4 However, theisk of priapism with testosterone replacementherapy in patients with Klinefelter syndromehould be kept in mind.

REFERENCES1. Zargooshi J: Priapism as a complication of high dose

estosterone therapy in a man with hypogonadism. J Urol 163:07, 2000.2. Shergill IS, Pranesh N, Hamid R, et al: Testosterone induced

riapism in Kallmann’s syndrome. J Urol 169: 1089, 2003.3. Whalen RK, Whitcomb RW, Crowley WFJ, et al: Pria-

ism in hypogonadal men receiving gonadotropin releasingormone. J Urol 145: 1051–1052, 1991.4. Lanfranco F, Kamischke A, Zitzmann M, et al: Klinefelter’s

yndrome. Lancet 364: 273–283, 2004.

UROLOGY 67 (3), 2006