Endocrine Abstracts

Case: A 34 year old was reviewed in reproductive clinic for gonadotropins therapy prior to micro-TESE. He was diagnosed with Klinefelter syndrome in his 20s in another hospital. Testosterone replacement was initiated with Nebido injection. However, he developed polycythemia, DVT and PE in 2013 and therefore the testosterone replacement was stopped. He had thrombophilia screen that showed heterozygous prothrombin gene mutation. Thereafter, he received anticoagulation for few months and was maintained on testosterone gel replacement 50 mg three times a week. Examination showed height of 184 cm. No eunuchoid body habitus but sparse body hair. Testicular volume was 2 cm on each side. On gel, his blood results showed serum testosterone level 19.5 nmol/l with FSH level of 36.5 unit/l and LH level of 10.8 unit/l and Hct of 47%. With view of his increased risk of thromboembolism, we agreed on cautious introduction of gonadotropins and withdrawal of Testogel to optimise any sperm production prior to surgical sperm retrieval by the urologist. From mid-august 2018, he was started on Menopur 75 units and Gonasi 2500 units to be taken twice weekly subcutaneously each. Five weeks on to therapy, he developed DVT eight days after flight to Canada. He was reviewed few weeks after the event in the clinic (November 2018), when he was off gonadotropins for few weeks and was already on anticoagulation (Apixaban 5 mg twice daily). Understandably, he was having hypo-gonadal symptoms with low testosterone level of 2.7 nmole/l.

Management: Along with his anticoagulation, he was restarted again on Menopur 75 units twice weekly with Gonasi third vial (~1500 IU twice a week). It was expected to have modest increase in testosterone level. Four weeks later, the testosterone level was still 2.8 nmol/l and therefore Gonasi increased to 2500 units twice daily plus continuing the Menopur 75 units twice a week. Six weeks later, his testosterone level was 4 nmol/l with Hct of 44.6%. At this point we agreed to increase his Menopure to 150 units twice weekly and keep the same dose of Gonasi in order to avoid increase risk of DVT. He was referred back to urologist for sperm retrieval with haematology consultation prior to the procedure. He is waiting to be seen.

Discussion Points:: • The increase risk of thromboembolism in patient with Klinefelter syndrome

• The challenge of spermatogenesis induction in patients with underlying risk of thromboembolism.

• The role of Gonadotropines therapy prior to micro-TESE.