02532nas a2200301   4500008004100000020001400041024001900055245011200074210006900186260010200255300001100357490000800368520147700376653003801853653005001891653002401941653004301965653001002008653003502018653004002053653001102093653002402104653001102128100001402139700001502153700001702168856004502185       1999                        eng d  a1516-3180  a100897261, dio00aDelayed hemolytic transfusion reaction presenting as a painful crisis in a patient with sickle cell anemia.0 aDelayed hemolytic transfusion reaction presenting as a painful c  aBrazilbFabron,A Jr. Universidade Federal de Sao Paulo/Escola Paulista de Medicina, Brazil.c1999  a38 - 90 v1173 aCONTEXT: Patients with sickle cell anemia (SCA) are frequently transfused with red blood cells (RBC). Recently we reported that the calculated risk of RBC alloimmunization per transfussed unit in Brazilian patients with SCA is 1.15%. We describe a delayed hemolytic transfusion reaction (DHTR) presenting as a painful crisis in a patient with SCA., CASE REPORT: A 35-year-old Brazilian female with homozygous SCA was admitted for a program of partial exchange transfusion prior to cholecystectomy. Her blood group was O RhD positive and no atypical RBC alloantibody was detected using the indirect antiglobulin technique. Pre-transfusional hemoglobin (Hb) was 8.7 g/dL and isovolumic partial exchange transfusion was performed using 4 units of ABO compatible packed RBC. Five days after the last transfusion she developed generalized joint pain and fever of 39 degrees C. Her Hb level dropped from 12.0 g/dL to 9.3 g/dL and the unconjugated bilirrubin level rose to 27 mmol/L. She was jaundiced and had hemoglobinuria. Hemoglobin electrophoresis showed 48.7% HbS, 46.6% HbA1, 2.7% HbA2, and 2.0% HbF. The patient's extended RBC phenotype was CDe, K-k+, Kp(a-b+), Fy(a-b-), M+N+s+, Le(a+b-), Di(a-). An RBC alloantibody with specificity to the Rh system (anti-c, titer 1:16.384) was identified by the indirect antiglobulin test. The Rh phenotype of the RBC used in the last packed RBC transfusion was CcDEe. The patient was discharged, asymptomatic, 7 days after admission.10a*Anemia, Sickle Cell/th [Therapy]10a*Erythrocyte Transfusion/ae [Adverse Effects]10a*Pain/et [Etiology]10aABO Blood-Group System/im [Immunology]10aAdult10aAnemia, Sickle Cell/bl [Blood]10aAnemia, Sickle Cell/im [Immunology]10aFemale10aFever/et [Etiology]10aHumans1 aFabron, A1 aMoreira, G1 aBordin, J, O  uhttps://notifylibrary.org/reference/4403