Hemoglobinopathies and coagulopathies are blood disorders that impair normal blood function, each with distinct mechanisms and clinical implications. Hemoglobinopathies, such as sickle cell disease (SCD), result from abnormal hemoglobin that can cause vascular obstructions, leading to both systemic and ocular complications. SCD, the most prevalent hemoglobinopathy, is marked by mutations that cause red blood cells to adopt a sickle shape under low oxygen conditions, increasing the risk of vaso-occlusion and serious complications like proliferative retinopathy. Coagulopathies, in contrast, affect the blood’s clotting ability, leading to either excessive bleeding or thrombosis. Disorders such as disseminated intravascular coagulation (DIC), HELLP syndrome, and autoimmune thrombocytopenia can result in severe systemic effects as well as ocular manifestations, including retinal hemorrhages, serous retinal detachment, and vascular occlusions. Timely diagnosis and treatment are essential in managing both hemoglobinopathies and coagulopathies to prevent severe complications, including vision loss.

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Hemoglobinopathies and Coagulopathies

  • Gustavo Sakuno,
  • Juliana Mika Kato,
  • Pedro C. Carricondo

摘要

Hemoglobinopathies and coagulopathies are blood disorders that impair normal blood function, each with distinct mechanisms and clinical implications. Hemoglobinopathies, such as sickle cell disease (SCD), result from abnormal hemoglobin that can cause vascular obstructions, leading to both systemic and ocular complications. SCD, the most prevalent hemoglobinopathy, is marked by mutations that cause red blood cells to adopt a sickle shape under low oxygen conditions, increasing the risk of vaso-occlusion and serious complications like proliferative retinopathy. Coagulopathies, in contrast, affect the blood’s clotting ability, leading to either excessive bleeding or thrombosis. Disorders such as disseminated intravascular coagulation (DIC), HELLP syndrome, and autoimmune thrombocytopenia can result in severe systemic effects as well as ocular manifestations, including retinal hemorrhages, serous retinal detachment, and vascular occlusions. Timely diagnosis and treatment are essential in managing both hemoglobinopathies and coagulopathies to prevent severe complications, including vision loss.