Kalam Sabrina, Beale Rupert, Hughes Derralynn, Kulasekararaj Austin, Srirangalingam Umasuthan
Department of Neurology, Charing Cross Hospital, London, W6 8RF, UK.
Department of Nephrology, Royal Free Hospital, London NW3 2QG, UK.
Oxf Med Case Reports. 2020 Mar 30;2020(3):omz125. doi: 10.1093/omcr/omz125. eCollection 2020 Mar.
Autoimmune haemolytic anaemia (AIHA) and paroxysmal nocturnal haemoglobinuria (PNH) are two distinct causes of haemolytic anaemia. They have different mechanisms that underpin their pathogenesis and, therefore, require different treatment strategies. The direct antiglobulin test (DAT) or Coombs test is positive in cases of immune-mediated haemolytic anaemia and, thus, is positive in AIHA but negative in PNH. We report a case of a woman presenting with a haemolytic anaemia who was found to have concomitant evidence of AIHA and PNH. The case highlights the importance of carrying out a comprehensive haemolysis work-up in patients who present with haemolytic anaemia.
自身免疫性溶血性贫血(AIHA)和阵发性睡眠性血红蛋白尿(PNH)是溶血性贫血的两种不同病因。它们具有不同的发病机制,因此需要不同的治疗策略。直接抗球蛋白试验(DAT)或库姆斯试验在免疫介导的溶血性贫血中呈阳性,因此在AIHA中呈阳性而在PNH中呈阴性。我们报告一例患有溶血性贫血的女性病例,该病例同时存在AIHA和PNH的证据。该病例凸显了对溶血性贫血患者进行全面溶血检查的重要性。