Abstract
Thalassemia is a hematologic disorder caused by genetic mutation resulting in impaired hemoglobin chain production. Patients with thalassemia commonly experience complications such as anemia, blood transfusion-related issues, hepatic or cardiac involvement, and psychosocial impacts. Rarely, priapism has been associated with thalassemia as an initial presentation or subsequently occurring at any time in the disease course. Our literature review summarizes the reported cases of thalassemia-associated priapism and delves into underlying mechanisms of its pathophysiology and appropriate management.
Highlights
Thalassemia is a hematologic disorder caused by mutations in the genes coding for hemoglobin chains
Endocrine complications of thalassemia major commonly involve growth hormone and insulin-like growth factor (IGF-1) axis resulting in IGF-1 deficiency associated with growth hormone deficiency [4]
Adrenal abnormalities in patients with thalassemia were appropriately identified with low-dose adrenocorticotropic hormone (ACTH) test rather than standard dose ACTH, deeming it necessary to perform a low-dose ACTH test to identify possible adrenal insufficiency or underlying latent hypocortisolism, especially in thalassemia patients undergoing major surgical procedures [5,6]
Summary
Thalassemia is a hematologic disorder caused by mutations in the genes coding for hemoglobin chains. Thalassemic patients with priapism as a complication or presenting concern experience further worsening of quality of life due to impairment of sexual function and the possible risk of penile fibrosis and, subsequently, permanent erectile dysfunction. In adolescents and young male patients affected with chronic disease (e.g., hemoglobinopathies, failure of pubertal growth, absence or delay of sexual development), infertility and sexual dysfunction are well-established disturbances secondary to hypogonadism and impaired spermatogenesis [19] Long-term complications of priapism in thalassemic individuals include penile fibrosis and erectile dysfunction, with discernable negative impacts on quality of life due to impaired sexual function and troublesome reproductive issues. A recent case reported treatment of erectile dysfunction in thalassemic men with the administration of transurethral E1-prostaglandins such as alprostadil as an effective, noninvasive therapy [25]
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