Andersen Frank D, d'Amore Francesco, Nielsen Finn Cilius, van Solinge Wouter, Jensen Finn, Jensen Peter D
Department of Hematology, Aarhus University Hospital, Aarhus, Denmark.
Hematol J. 2004;5(6):543-5. doi: 10.1038/sj.thj.6200556.
Iron overload is a serious condition, which may lead to irreversible organ damage. The risk of iron accumulation in pyruvate kinase deficiency (PKD) has traditionally been regarded as low, but recent evidence has questioned this notion. We here present a case of a young PKD patient showing evidence of asymptomatic iron accumulation measured as liver iron concentration (LIC) obtained noninvasively by magnetic resonance imaging. The iron overload was not related to blood transfusions, but rather secondary to concomitant risk factors leading to increased intestinal iron absorption, such as chronic hemolysis and splenectomy. The iron status of PKD patients, preferably assessed by LIC measurements, should therefore be evaluated regularly also in asymptomatic patients. This evaluation should start already at a young age, in order to initiate iron chelation before the development of iron-induced organ damage.
铁过载是一种严重的病症，可能导致不可逆的器官损伤。传统上认为丙酮酸激酶缺乏症（PKD）中铁蓄积的风险较低，但最近的证据对这一观点提出了质疑。我们在此报告一例年轻的PKD患者，其通过磁共振成像无创获得的肝脏铁浓度（LIC）显示有无症状铁蓄积的证据。铁过载与输血无关，而是继发于导致肠道铁吸收增加的伴随风险因素，如慢性溶血和脾切除术。因此，PKD患者的铁状态，最好通过LIC测量来评估，即使是无症状患者也应定期进行评估。这种评估应该在患者年轻时就开始，以便在铁诱导的器官损伤发生之前开始铁螯合治疗。
