H Wan, CK Li, TF Fok

Abstract

We describe a case of paroxysmal cold haemaglobinuria (PCH) in a 23-month-old girl who presented with passage of dark urine, fever, chills and rigors six days after an episode of upper respiratory tract infection caused by respiratory syncytial virus. Paroxysmal cold haemaglobinuria is a rare form of autoimmune anaemia occurring in children. The diagnosis is confirmed by a positive Donath-Landsteiner test. The course of the haemolysis is usually self-limiting but the degree of anaemia thus caused can be severe. Treatment is symptomatic only and may involve blood transfusion if there is evidence of cardiopulmonary compromise.

我們闡述一例 23 個月大女孩的陣發性寒冷性血紅蛋白尿，其臨床表現為呼吸道合胞病毒引起的上呼吸道感染後，排深色小便、發熱、發冷和寒顫。陣發性寒冷性血紅蛋白尿是兒童自身免疫性貧血中的一種罕見類型。其診斷可由冷熱溶血試驗陽性而確診。溶血的過程通常為自限性，但貧血的程度卻可以是嚴重的。治療方面只是對症和當危及心肺功能時給予輸血治療。