Germ Cell Dysfunction is Universal in Adolescent Male Patients with β-thalassemia Following Earlier Successful Hematopoietic Stem Cell Transplantation
Issued Date
2025-05-27
Resource Type
eISSN
13085735
Scopus ID
2-s2.0-105007117180
Pubmed ID
39535107
Journal Title
Journal of Clinical Research in Pediatric Endocrinology
Volume
17
Issue
2
Start Page
136
End Page
145
Rights Holder(s)
SCOPUS
Bibliographic Citation
Journal of Clinical Research in Pediatric Endocrinology Vol.17 No.2 (2025) , 136-145
Suggested Citation
Piriyapokin N., Mahachoklertwattana P., Poomthavorn P., Anurathapan U., Chiangjong W. Germ Cell Dysfunction is Universal in Adolescent Male Patients with β-thalassemia Following Earlier Successful Hematopoietic Stem Cell Transplantation. Journal of Clinical Research in Pediatric Endocrinology Vol.17 No.2 (2025) , 136-145. 145. doi:10.4274/jcrpe.galenos.2024.2024-6-5 Retrieved from: https://repository.li.mahidol.ac.th/handle/123456789/110589
Title
Germ Cell Dysfunction is Universal in Adolescent Male Patients with β-thalassemia Following Earlier Successful Hematopoietic Stem Cell Transplantation
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Abstract
Objective: To assess gonadal function in adolescent male patients with β-thalassemia who underwent earlier successful hematopoietic stem cell transplantation (HSCT). Methods: Fifty-two male patients with β-thalassemia, aged ≥10 years, who had undergone HSCT ≥2 years previously were included. Clinical data, such as age, genital Tanner (GT) stage at HSCT and enrollment, and serum ferritin levels, were collected. Gonadal function was evaluated through measurements of serum luteinizing hormone, follicle-stimulating hormone (FSH), testosterone, inhibin B levels, and semen analysis. Results: Age at enrollment and HSCT were 17 (10-31) and 9 (1-19) years, respectively. The duration from HSCT to enrollment was 7.5 (2-20) years. Of 52 patients, 46 (88%) exhibited Sertoli cell dysfunction. Thirty-one patients had relatively small testes for their GT stage, 34 of 44 with GT V had elevated FSH of ≥5 IU/L, and 20 of 49 with GT stages 2-5 had low serum inhibin B levels. None of the patients with GT stage 5 showed Leydig cell dysfunction or gonadotropin deficiency. Serum FSH ≥8 IU/L showed the best diagnostic accuracy for detecting oligo- and azoo-spermia. All 39 patients who underwent semen analysis had >1 abnormal parameters. Having relatively small testes for GT stage and serum FSH ≥8 IU/L were associated with oligospermia or azoospermia (p<0.01). Conclusion: Male patients with β-thalassemia after HSCT experienced universal impaired spermatogenesis and frequent Sertoli cell dysfunction but their Leydig cell function appeared to be preserved. Male patients and/or their guardians should be informed of the high likelihood of future subfertility before HSCT.