Dahlberg Pia, Bartfay Sven-Erik, Karason Kristjan, Mellqvist Ulf-Henrik, Bollano Entela
Sahlgrenska universitetssjukhuset - Kardiologen Göteborg, Sweden Sahlgrenska universitetssjukhuset - Goteborg, Sweden.
Sahlgrenska universitetssjukhuset - Kardiologen Göteborg, Sweden Sahlgrenska universitetssjukhuset - Cardiology Gothenburg, Sweden.
Lakartidningen. 2018 Mar 26;115:EY3F.
Amyloidosis is a disease complex characterized by the deposition of protein fibrils in various tissues, which leads to structural and functional derangement of the affected organ. There are different types of amyloidosis categorized on the basis of the type of protein fibrils deposited. Cardiac involvement has been predominantly noted in amyloid light chain (AL) amyloidosis and is the major prognostic determinant and influences the therapeutic strategy. In AL amyloidosis, heart transplantation is generally not recommended because of a high risk of recurrence in the transplanted heart and poor survival rate. However, a favourable outcome can be achieved if heart transplantation is followed by an autologous stem cell transplantation (ASCT). We describe our experience from the two first patients with AL amyloidosis treated with heart transplantation and subsequent ASCT at Sahlgrenska University Hospital.
淀粉样变性是一种复杂的疾病，其特征是蛋白质原纤维在各种组织中沉积，导致受影响器官的结构和功能紊乱。根据沉积的蛋白质原纤维类型，淀粉样变性有不同的类型。心脏受累主要见于轻链（AL）淀粉样变性，是主要的预后决定因素，并影响治疗策略。在AL淀粉样变性中，由于移植心脏复发风险高和生存率低，一般不建议进行心脏移植。然而，如果心脏移植后进行自体干细胞移植（ASCT），则可以取得良好的效果。我们描述了在萨尔格伦斯卡大学医院对首例两名接受心脏移植及后续ASCT治疗的AL淀粉样变性患者的治疗经验。
