Gonadal dysfunction in adult male patients with thalassemia major: an update for clinicians caring for thalassemia

Objectives: Endocrine disorders continue to affect the health of thalassemia patients, foremost of which is hypogonadism being the most frequent endocrine complication that involves 70-80% of beta-thalassemia major (β-TM) patients. Actually, the role of iron overload in endocrine complications is well known. Our study goals were to investigate gonadal function, assess pubertal status among Syrian male patients with β-TM and correlate hormonal panel with serum ferritin as the marker of iron overload. Methods: 56 β-TM regularly transfused male patients were enrolled in this study, they were 21.91±5.01 years old. FSH, LH, Total Testosterone, and Serum Ferritin were measured for all patients, 52 of them undergone pubertal status evaluation. Results: Results showed that 60.7% of patients suffered from hypogonadism, which was hypogonadotropic hypogonadism in 97.06% of them. Delayed puberty was seen in 7.7% of the patients, while arrested puberty was found in 82.69% of them. All patients had iron overload and 92.86% of them suffered from severe iron elevation. Both gonadal and pubertal status were independent of the serum ferritin levels (P=0.73), (P=0.81) respectively. There was significant positive correlation between FSH: LH (r=0.584, P=0.0001), FSH: Testosterone (r=0.562, P=0.0001), LH: Testosterone (r=0.746, P=0.0001), MCHC: Testosterone (r=0.292, P=0.038), and BMI: Hb (r=0.351, P=0.009). Conclusions: Our findings indicated that hypogonadism, arrested puberty and severe iron overload were highly prevalent among male patients with β-TM. Patients with better gonadal reserve have higher BMI than those with gonadal dysfunction. We suggest that hypogonadism in β-TM patients is not directly related to serum ferritin levels; other potential factors (such as chronic anemia, hypoxia, and genetic predisposition) may contribute. Also we suggest that adequate blood transfusion and appropriate iron chelation, along with regular evaluation for gonadal status and timely intervention can improve the management of aforementioned complications, thus ameliorating patients’ quality of life.

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Body Composition in Adult Patients with Thalassemia Major

International Journal of Endocrinology ◽

10.1155/2016/6218437 ◽

2016 ◽

Vol 2016 ◽

pp. 1-7 ◽

Cited By ~ 2

Author(s):

Marianna Vlychou ◽

Evangelos Alexiou ◽

Paschalis Thriskos ◽

Ioannis Fezoulidis ◽

Katerina Vassiou

Keyword(s):

Body Composition ◽

Adult Male ◽

Lean Mass ◽

Thalassemia Major ◽

Dual Energy ◽

Adult Patients ◽

Whole Body ◽

Male And Female ◽

X Ray ◽

Male Patients

Objective. To assess body composition in adult male and female patients with thalassemia major by dual-energy X-ray absorptiometry (DXA) and to compare the findings with a group of healthy age-matched controls. Methods. Our study group included sixty-two patients (27 males, mean age 36 years, and 35 females, mean age 36.4 years) and fifteen age-matched healthy controls. All patients had an established diagnosis of thalassemia major and followed a regular blood transfusion scheme since childhood and chelation treatment. Fat, lean, and bone mineral density (BMD) were assessed with dual-energy X-ray absorptiometry. Ferritin levels and body mass index of all patients and controls were also recorded. Student t-test and Wilcoxon test were performed and statistical significance was set at p<0.05. Results. BMD and whole body lean mass are lower in both male and female adult patients compared with controls (p<0.01 in both groups), whereas whole body fat mass was found to have no statistically significant difference compared to controls. Regional trunk fat around the abdomen was found to be lower in male patients compared to controls (p=0.02). Conclusion. Severe bone loss and diminished lean mass are expected in adult male and female patients with thalassemia major. Fat changes seem to affect mainly male patients.

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