Department of Pediatrics, Division of Pediatric Hematology/Oncology, Oregon Health & Science University, 3181 Southwest Sam Jackson Park Road, Mail Code CDRCP, Portland, OR 97239, USA.
Med Clin North Am. 2017 Mar;101(2):361-374. doi: 10.1016/j.mcna.2016.09.008. Epub 2016 Dec 8.
Red blood cell (RBC) destruction can be secondary to intrinsic disorders of the RBC or to extrinsic causes. In the congenital hemolytic anemias, intrinsic RBC enzyme, RBC membrane, and hemoglobin disorders result in hemolysis. The typical clinical presentation is a patient with pallor, anemia, jaundice, and often splenomegaly. The laboratory features include anemia, hyperbilirubinemia, and reticulocytosis. For some congenital hemolytic anemias, splenectomy is curative. However, in other diseases, avoidance of drugs and toxins is the best therapy. Supportive care with transfusions are also mainstays of therapy. Chronic hemolysis often results in the formation of gallstones, and cholecystectomy is often indicated.
红细胞（RBC）破坏可继发于 RBC 的内在紊乱或外在原因。在先天性溶血性贫血中，RBC 酶、RBC 膜和血红蛋白内在紊乱导致溶血。典型临床表现为苍白、贫血、黄疸，常伴有脾肿大。实验室特征包括贫血、高胆红素血症和网织红细胞增多。对于一些先天性溶血性贫血，脾切除术是治愈性的。然而，在其他疾病中，避免药物和毒素是最佳治疗方法。输血等支持性治疗也是治疗的基础。慢性溶血常导致胆石形成，常需要行胆囊切除术。
