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, Ashraf T. Soliman, Heba Elsedfy, Alice Albu, Soad Al Jaouni, Salvatore Anastasi, Maria Grazia Bisconte, Duran Canatan, Soteroula Christou, Shahina Daar, Salvatore Di Maio, Mohamed El Kholy, Doaa Khater, Mohamed Elshinawy, Yurdanur Kilinc, Roberto Mattei, Hala H. Mosli, Alessandra Quota, Maria Grazia Roberti, Praveen SobtiSaif A L Yaarubi, Saveria Canpisi, Christos Kattamis

Research output: Contribution to journalReview article

4 Citations (Scopus)

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.

Original languageEnglish
Article numbere2017001
JournalMediterranean Journal of Hematology and Infectious Diseases
Volume9
Issue number1
DOIs
Publication statusPublished - 2017

Fingerprint

Thalassemia
Hypogonadism
beta-Thalassemia
Hormone Replacement Therapy
Progestins
Adolescent Medicine
Iron Overload
Therapeutics
Patient Compliance
Oral Contraceptives
Sex Characteristics
Comorbidity
Liver Diseases
Anemia
Estradiol
Estrogens
Chronic Disease
Steroids
Physicians
Surveys and Questionnaires

Keywords

  • Benefits and disadvantages
  • Hormone replacement therapy
  • Hypogonadism
  • Thalassemia

ASJC Scopus subject areas

  • Hematology
  • Infectious Diseases

Cite this

Review and recommendations on management of adult female thalassemia patients with hypogonadism based on literature review and experience of ICET-A network specialists. / De Sanctis, Vincenzo; Soliman, Ashraf T.; Elsedfy, Heba; Albu, Alice; Al Jaouni, Soad; Anastasi, Salvatore; Bisconte, Maria Grazia; Canatan, Duran; Christou, Soteroula; Daar, Shahina; Di Maio, Salvatore; El Kholy, Mohamed; Khater, Doaa; Elshinawy, Mohamed; Kilinc, Yurdanur; Mattei, Roberto; Mosli, Hala H.; Quota, Alessandra; Roberti, Maria Grazia; Sobti, Praveen; Yaarubi, Saif A L; Canpisi, Saveria; Kattamis, Christos.

In: Mediterranean Journal of Hematology and Infectious Diseases, Vol. 9, No. 1, e2017001, 2017.

Research output: Contribution to journalReview article

De Sanctis, V, Soliman, AT, Elsedfy, H, Albu, A, Al Jaouni, S, Anastasi, S, Bisconte, MG, Canatan, D, Christou, S, Daar, S, Di Maio, S, El Kholy, M, Khater, D, Elshinawy, M, Kilinc, Y, Mattei, R, Mosli, HH, Quota, A, Roberti, MG, Sobti, P, Yaarubi, SAL, Canpisi, S & Kattamis, C 2017, 'Review and recommendations on management of adult female thalassemia patients with hypogonadism based on literature review and experience of ICET-A network specialists', Mediterranean Journal of Hematology and Infectious Diseases, vol. 9, no. 1, e2017001. https://doi.org/10.4084/MJHID.2017.001
De Sanctis, Vincenzo ; Soliman, Ashraf T. ; Elsedfy, Heba ; Albu, Alice ; Al Jaouni, Soad ; Anastasi, Salvatore ; Bisconte, Maria Grazia ; Canatan, Duran ; Christou, Soteroula ; Daar, Shahina ; Di Maio, Salvatore ; El Kholy, Mohamed ; Khater, Doaa ; Elshinawy, Mohamed ; Kilinc, Yurdanur ; Mattei, Roberto ; Mosli, Hala H. ; Quota, Alessandra ; Roberti, Maria Grazia ; Sobti, Praveen ; Yaarubi, Saif A L ; Canpisi, Saveria ; Kattamis, Christos. / Review and recommendations on management of adult female thalassemia patients with hypogonadism based on literature review and experience of ICET-A network specialists. In: Mediterranean Journal of Hematology and Infectious Diseases. 2017 ; Vol. 9, No. 1.
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title = "Review and recommendations on management of adult female thalassemia patients with hypogonadism based on literature review and experience of ICET-A network specialists",
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 = "Benefits and disadvantages, Hormone replacement therapy, Hypogonadism, Thalassemia",
author = "{De Sanctis}, Vincenzo and Soliman, {Ashraf T.} and Heba Elsedfy and Alice Albu and {Al Jaouni}, Soad and Salvatore Anastasi and Bisconte, {Maria Grazia} and Duran Canatan and Soteroula Christou and Shahina Daar and {Di Maio}, Salvatore and {El Kholy}, Mohamed and Doaa Khater and Mohamed Elshinawy and Yurdanur Kilinc and Roberto Mattei and Mosli, {Hala H.} and Alessandra Quota and Roberti, {Maria Grazia} and Praveen Sobti and Yaarubi, {Saif A L} and Saveria Canpisi and Christos Kattamis",
year = "2017",
doi = "10.4084/MJHID.2017.001",
language = "English",
volume = "9",
journal = "Mediterranean Journal of Hematology and Infectious Diseases",
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TY - JOUR

T1 - Review and recommendations on management of adult female thalassemia patients with hypogonadism based on literature review and experience of ICET-A network specialists

AU - De Sanctis, Vincenzo

AU - Soliman, Ashraf T.

AU - Elsedfy, Heba

AU - Albu, Alice

AU - Al Jaouni, Soad

AU - Anastasi, Salvatore

AU - Bisconte, Maria Grazia

AU - Canatan, Duran

AU - Christou, Soteroula

AU - Daar, Shahina

AU - Di Maio, Salvatore

AU - El Kholy, Mohamed

AU - Khater, Doaa

AU - Elshinawy, Mohamed

AU - Kilinc, Yurdanur

AU - Mattei, Roberto

AU - Mosli, Hala H.

AU - Quota, Alessandra

AU - Roberti, Maria Grazia

AU - Sobti, Praveen

AU - Yaarubi, Saif A L

AU - Canpisi, Saveria

AU - Kattamis, Christos

PY - 2017

Y1 - 2017

N2 - 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.

AB - 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.

KW - Benefits and disadvantages

KW - Hormone replacement therapy

KW - Hypogonadism

KW - Thalassemia

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M3 - Review article

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JO - Mediterranean Journal of Hematology and Infectious Diseases

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