A Rare Presentation of Transfusional Hemochromatosis: Hypogonadotropic Hypogonadism.
Hemochromatosis is a disease caused by extraordinary iron deposition in parenchymal cells leading to cellular damage and organ dysfunction. β-thalassemia major is one of the causes of secondary hemochromatosis due to regular transfusional treatment for maintaining adequate levels of hemoglobin. Hypogonadism is one of the potential complications of hemochromatosis, usually seen in patients with a severe iron overload, and it shows an association with diabetes and cirrhosis in adult patients. We describe a patient with mild transfusional hemochromatosis due to β-thalassemia major, presenting with central hypogonadism in the absence of cirrhosis or diabetes. Our case showed an atypical presentation with hypogonadotropic hypogonadism without severe hyperferritinemia, cirrhosis, or diabetes. With this case, we aim to raise awareness of hypogonadotropic hypogonadism in patients with intensive transfused thalassemia major even if not severe hemochromatosis so that hypogonadism related complications, such as osteoporosis, anergia, weakness, sexual dysfunction, and infertility, could be more effectively managed in these patients.
Department of Endocrinology and Metabolism, Medical Faculty, Yuzuncu Yil University, 65080 Van, Turkey.
Rifki
R
Ucler
Department of Hematology, Medical Faculty, Yuzuncu Yil University, 65080 Van, Turkey.
Erdal
E
Kara
Department of Endocrinology and Metabolism, Medical Faculty, Yuzuncu Yil University, 65080 Van, Turkey.
Murat
M
Atmaca
Department of Gastroenterology, Medical Faculty, Yuzuncu Yil University, 65080 Van, Turkey.
Sehmus
S
Olmez
Department of Endocrinology and Metabolism, Medical Faculty, Yuzuncu Yil University, 65080 Van, Turkey.
Murat
M
Alay
Department of Internal Medicine, Medical Faculty, Yuzuncu Yil University, 65080 Van, Turkey.
Yaren
Y
Dirik
Department of Radiology, Medical Faculty, Yuzuncu Yil University, 65080 Van, Turkey.
Aydin
A
Bora