de Ruiter E D, Baggen M G A, Middelkoop M P
Department of Internal Medicine, Ikazia Hospital, Rotterdam, The Netherlands.
Neth J Med. 2003 May;61(5):174-6.
Paroxysmal nocturnal haemoglobinuria (PNH) is an acquired haemopoietic stem cell disorder characterised clinically by chronic haemolytic anaemia with acute episodes, thrombosis and bone marrow failure. It is a rare condition, which usually occurs in younger people. Immunophenotyping and flow cytometry play a key role in diagnosing PNH. Treatment is mainly supportive. Because it is so rare, delay in diagnosis is not uncommon in patients with PNH, which has a considerable impact on patient management and prognosis. We present this case to draw attention to this rare cause of haemolytic anaemia, which should be considered in any patient, of any age, who has signs of chronic haemolysis.
阵发性睡眠性血红蛋白尿症(PNH)是一种获得性造血干细胞疾病,临床特征为慢性溶血性贫血伴急性发作、血栓形成和骨髓衰竭。这是一种罕见疾病,通常发生于年轻人。免疫表型分析和流式细胞术在PNH的诊断中起关键作用。治疗主要是支持性的。由于该病非常罕见,PNH患者诊断延迟的情况并不少见,这对患者的管理和预后有相当大的影响。我们展示此病例以引起对这种罕见的溶血性贫血病因的关注,任何年龄有慢性溶血迹象的患者都应考虑这一病因。