Área de Investigación en Medicina Interna, Servicio de Clínica Médica, Hospital Italiano de Buenos Aires.
Servicio de Cardiología, Hospital Italiano de Buenos Aires.
Arch Cardiol Mex. 2023;93(4):435-441. doi: 10.24875/ACM.22000208.
To describe the evolution of serum free light chains (FLC) in the period between orthotopic heart transplantation (OHT) and autologous stem cell transplantation (ASCT), the hematological response one year after ASCT and chemotherapy and immunosuppressive treatment in patients with AL amyloidosis.
Case series of consecutive patients diagnosed with AL amyloidosis who received OHT followed by ASCT from the Institutional Registry of Amyloidosis of the Italian Hospital of Buenos Aires, between January 2010 and November 2021. FLC values between transplants and at year post ASCT. Quantitative variables were described with their median and interquartile range. Categorical variables as absolute and relative frequencies.
Of 106 patients with AL amyloidosis, 6 had an OHT followed by ASCT. The median age was 55 years. Most were men (n = 5). In the period between transplants, the involved CLL decreased in two patients and remained stable in three. All achieved complete hematologic remission 1 year after ASCT. A single patient presented relapse in the transplanted solid organ. Tacrolimus, mycophenolate mofetil, and corticosteroids were the immunosuppressive regimen used after OHT.
OHT represents a treatment option in patients with severe heart failure due to amyloidosis, allowing later intensive treatment with induction chemotherapy and ASCT. Although studies are lacking, immunosuppressive therapy after OHT might have some effect on clonal plasma cells.
描述原发性心脏移植（OHT）和自体干细胞移植（ASCT）之间、ASCT 后一年及化疗和免疫抑制治疗期间，AL 淀粉样变性患者血清游离轻链（FLC）的演变情况。
这是一项来自布宜诺斯艾利斯意大利医院淀粉样变性机构注册中心的连续病例系列研究，纳入了 2010 年 1 月至 2021 年 11 月期间接受 OHT 后行 ASCT 的 AL 淀粉样变性患者。描述了移植前后及 ASCT 后 1 年的 FLC 值。定量变量采用中位数和四分位距表示。分类变量采用绝对和相对频率表示。
在 106 例 AL 淀粉样变性患者中，有 6 例行 OHT 后 ASCT。中位年龄为 55 岁，大多数为男性（n=5）。在移植期间，2 例受累的 CLL 减少，3 例稳定。所有患者在 ASCT 后 1 年均获得完全血液学缓解。1 例移植实体器官患者复发。OHT 后使用的免疫抑制方案为他克莫司、霉酚酸酯和皮质类固醇。
OHT 是严重淀粉样变性导致心力衰竭患者的一种治疗选择，允许随后进行强化诱导化疗和 ASCT。尽管缺乏研究，但 OHT 后的免疫抑制治疗可能对克隆浆细胞有一定影响。
