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Title: Delayed hemolytic transfusion reaction presenting as a painful crisis in a patient with sickle cell anemia
Authors: Fabron Junior, Antonio [UNIFESP]
Moreira Junior, Gilberto [UNIFESP]
Bordin, Jose Orlando [UNIFESP]
Universidade Federal de São Paulo (UNIFESP)
Keywords: Sickle cell anemia
Transfusion reaction
Painful crisis
Issue Date: 7-Jan-1999
Publisher: Associação Paulista de Medicina - APM
Citation: São Paulo Medical Journal. Associação Paulista de Medicina - APM, v. 117, n. 1, p. 38-39, 1999.
Abstract: CONTEXT: 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 transfused 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°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.
CONTEXTO: Pacientes com anemia falciforme recebem com freqüência concentrados de glóbulos vermelhos (CGV). Recentemente, nós relatamos que o risco de alo-imunização eritrocitária por unidade transfundida em pacientes brasileiros com anemia falciforme é 1,15%. RELATO DE CASO: Nós descrevemos uma reação transfusional hemolítica tardia (RTHT) apresentando-se como uma crise dolorosa em um paciente com anemia falciforme.
ISSN: 1516-3180
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