TY - JOUR T1 - The sickle cell hemolytic transfusion reaction syndrome. JF - Transfusion Y1 - 1997 A1 - Petz, L D A1 - Calhoun, L A1 - Shulman, I A A1 - Johnson, C A1 - Herron, R M KW - *Anemia, Sickle Cell/th [Therapy] KW - *Blood Transfusion/ae [Adverse Effects] KW - *Hemolysis KW - Adolescent KW - Adult KW - Aging/ph [Physiology] KW - Erythrocyte Aging KW - Erythropoiesis KW - Female KW - Hematocrit KW - Hemolysis/im [Immunology] KW - Humans KW - Male KW - Middle Aged KW - Reticulocyte Count KW - Syndrome KW - Time Factors AB - BACKGROUND: Patients with sickle cell anemia may develop serious, life-threatening hemolytic transfusion reactions (HTRs). More severe anemia may develop after the HTR than was present before transfusion, which suggests the possibility of an increased rate of hemolysis of autologous red cells., STUDY DESIGN AND METHODS: The signs and symptoms occurring during eight severe HTRs that occurred in five patients with sickle cell anemia were reviewed, as were published reports by other investigators. Calculations of red cell production and destruction incorporating known correction factors for reticulocyte maturation were performed to determine the most probable mechanism for the striking drop in hematocrit observed in several instances., RESULTS: A characteristic constellation of findings was recognized in some severe HTRs in patients with sickle cell anemia. Calculations of daily red cell production and senescence indicated that a marked drop in hematocrit occurs when erythropoiesis is suppressed in a patient with a short red cell life span and that this could account for severe posttransfusion anemia when donor red cells are hemolyzed during an HTR., CONCLUSION: A sickle cell HTR syndrome was defined. A rapid increase in the severity of anemia occurs in patients with sickle cell anemia when all donor red cells are hemolyzed during an HTR and when there is suppression of erythropoiesis, as commonly occurs as a result of transfusion or concomitant illness. Although an increased rate of hemolysis of autologous red cells may also occur, more definitive data are required to document that in these patients. M1 - wdn, 0417360 PB - Petz,L D. Division of Transfusion Medicine, University of California, Los Angeles 90095-1713, USA. CY - UNITED STATES VL - 37 SN - 0041-1132 CP - 4 N1 - Comment in: Transfusion. 1997 Apr;37(4):357-61; PMID: 9111271, Comment in: Transfusion. 1997 Oct;37(10):1098-9; author reply 1100-2; PMID: 9354833, Comment in: Transfusion. 1997 Oct;37(10):1099-100; author reply 1100-2; PMID: 9354834 L2 - http://ovidsp.ovid.com/ovidweb.cgi?T=JS&PAGE=reference&D=med4&NEWS=N&AN=9111275 ID - 4437 ER -