TY - JOUR
T1 - Congenital Hemolytic Anemia
AU - Haley, Kristina
N1 - Publisher Copyright:
© 2016 Elsevier Inc.
PY - 2017/3/1
Y1 - 2017/3/1
N2 - 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.
AB - 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.
KW - Aplastic crisis
KW - Hemolysis
KW - Jaundice
KW - Splenectomy
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U2 - 10.1016/j.mcna.2016.09.008
DO - 10.1016/j.mcna.2016.09.008
M3 - Review article
C2 - 28189176
AN - SCOPUS:85007578679
SN - 0025-7125
VL - 101
SP - 361
EP - 374
JO - Medical Clinics of North America
JF - Medical Clinics of North America
IS - 2
ER -