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Review
. 2017 Jan 1;9(1):e2017001.
doi: 10.4084/MJHID.2017.001. eCollection 2017.

Review and Recommendations on Management of Adult Female Thalassemia Patients with Hypogonadism based on Literature Review and Experience of ICET-A Network Specialists

Vincenzo De Sanctis  1 , Ashraf T Soliman  2 , Heba Elsedfy  3 , Alice Albu  4 , Soad Al Jaouni  5 , Salvatore Anastasi  6 , Maria Grazia Bisconte  7 , Duran Canatan  8 , Soteroula Christou  9 , Shahina Daar  10 , Salvatore Di Maio  11 , Mohamed El Kholy  3 , Doaa Khater  12 , Mohamed Elshinawy  13 , Yurdanur Kilinc  14 , Roberto Mattei  15 , Hala H Mosli  16 , Alessandra Quota  17 , Maria Grazia Roberti  18 , Praveen Sobti  19 , Saif Al Yaarubi  20 , Saveria Canpisi  21 , Christos Kattamis  22
Affiliations
Review

Review and Recommendations on Management of Adult Female Thalassemia Patients with Hypogonadism based on Literature Review and Experience of ICET-A Network Specialists

Vincenzo De Sanctis et al. Mediterr J Hematol Infect Dis. .

Abstract

Background: Multi-transfused thalassemia major (TM) patients frequently develop severe endocrine complications, mainly due to iron overload, anemia, and chronic liver disease, which require prompt diagnosis, treatment and follow-up by specialists. The most common endocrine complication documented is hypogonadotropic hypogonadism which increases with age and associated comorbidities. It is thus important for physicians to have a clear understanding of the pathophysiology and management of this disorder. Also to be aware of the side effects, contraindications and monitoring of sex steroid therapy. In this paper, practical ICET-A recommendations for the management of hypogonadism in adult females with TM are addressed.

Methods: In March 2015, the Coordinator of the International Network of Clinicians for Endocrinopathies in Thalassemia and Adolescent Medicine (ICET-A) conducted a two-step survey to assess the attitudes and practices of doctors in the ICET-A network taking care of adult female TM patients with hypogonadism. They were clinically characterized by the absence of pubertal development or discontinuation or regression of the maturation of secondary sex characteristics, and biochemically by persistent low FSH, LH and estradiol levels. Recently a supplementary survey on adult female hypogonadism in TM was undertaken within the ICET-A network.

Results: The completed questionnaires were returned by 16 of 27 specialists (59.2%) following 590 female TM patients over the age of 18 years; 315 patients (53.3%) had hypogonadism, and only 245 (74.6%) were on hormone replacement therapy (HRT). Contraceptive oral pills (COC) were the first treatment choice in 11 centers (68.7%). A wide range of COCs was used with different progestin contents. In general, the patients' compliance to treatment was reported as good in 81.2 % of centers. The frequency of required tests for follow-up HRT, in addition to the regular check-up for thalassemia, was variable in the participating centers.

Conclusions: Doctors taking care of TM patients should have sound knowledge of the pathophysiology of hypogonadism in adult females with TM. They should know the potential effects of HRT including advantages and disadvantages of estrogen and progestins. Moreover, they should keep in consideration the emotional needs of these patients dreaming of attaining a full pubertal development.

Keywords: Thalassemia; benefits and disadvantages; hormone replacement therapy; hypogonadism.

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References

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