Late diagnosis of sickle cell anemia in the course of liver crisis
Keywords:
hemolytic anemia, sickle cell disease, hemoglobinopathyAbstract
Introduction: Sickle cell disease is the most common congenital hemolytic anemia worldwide. In Cuba, since 1986, prenatal diagnosis has been carried out, which consists of the identification of the carrier woman (SA) in the first weeks of pregnancy and the study of her partner.
Objective: To present a case with a late diagnosis of sickle cell anemia in the course of a hepatic crisis.
Clinical case: A 30-year-old black male patient with suspected liver cirrhosis received at the "Iván Portuondo Hospital" in San Antonio de los Baños with recurrent abdominal pain of childhood onset and asthenia. Physical examination revealed icterus and globular abdomen, painful on palpation, and 4 cm hepatomegaly. The blood count showed severe anemia, moderate thrombocytopenia, elevated liver enzyme values, and direct predominance of hyperbilirubinemia. He presented worsening symptoms and hemodynamic instability for which he was transfused. Given the emergence of neurological manifestations, she was transferred to therapy. Despite the transfusion, he continued to have severe anemia, with reticulocytosis. Due to the deterioration of his general condition and the suspicion of hemoglobinopathy with a mixed hepatic crisis, it was decided to perform a total exchange transfusion to reduce the bilirubin levels. In addition, steroid therapy was initiated. Hemoglobin electrophoresis was performed, which confirmed the diagnosis of sickle cell anemia.
Conclusions: Sickle cell disease is the most common genetic disease in the world. If it is not diagnosed early, its complications can appear more frequently and intensely.
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