An Uncommon Display of the Sickle Cell Trait Coupled with the Beta-Thalassemia Trait as Hypersplenism

Abstract

Abstract A structural flaw in the globin gene causes hemoglobinopathies, while a flaw in the globin chain’s synthesis causes thalassemia. One of the most prevalent hemoglobinopathies worldwide is sickle cell disease. Any region of the body can be affected, but the spleen is one of the most often afflicted and the first organs to suffer damage in sickle cell anemia (SCA). Splenomegaly, splenic infarction, hypersplenism, and acute splenic sequestration are the most common manifestations. Usually enlarged in the first 10 years of life, it gradually atrophies and eventually requires an autosplenectomy. It is well established that splenic complications from SCA are linked to higher rates of morbidity and in some cases, even death. This case report discusses a unique presentation of a patient who has hypersplenism together with beta-thalassemia and sickle cell trait. This is a case report of the adult patient admitted in Dhiraj General Hospital, SBKS MIRC, Sumandeep Vidyapeeth, Waghodia, Piparia, Vadodara-391760. The high-performance liquid chromatography (HPLC) technique was used to diagnose thalassemia trait and sickle cell trait. The clinical, ultrasonographic and computed tomography scan methods have been used to identify splenomegaly. The patient had clinical symptoms suggestive of beta-thalassemia and sickle cell trait with hypersplenism. This was supported by his peripheral smear, ultrasonography, contrast-enhanced abdomen–pelvis, HPLC, and bone marrow biopsy. It is imperative that clinicians remain cognizant of this infrequent consequence. Hence, early diagnosis and genetic counseling are of great importance for improving the prognosis of these patients.