Division of Hematology/Oncology, Brookdale University Hospital Medical Center, Brooklyn, NY, USA.
Division of Hematology/Oncology, Brookdale University Hospital Medical Center, Brooklyn, NY, USA.
Crit Rev Oncol Hematol. 2017 Apr;112:103-112. doi: 10.1016/j.critrevonc.2017.02.011. Epub 2017 Feb 17.
Interferon (IFN) has been associated with development of thrombotic microangiopathy including thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). We reviewed literature from the earliest reported association in 1993, to July 2016 and found 68 cases. Analysis of this data shows: (1) Mean age at diagnosis was 47 years (95% CI, 44-50). (2) Majority of cases were seen where IFN was used for the treatment of chronic myelogenous leukemia (CML), multiple sclerosis (MS), chronic hepatitis C virus infection (HCV) and one case each for hairy cell leukemia (HCL) and Sezary syndrome. (3) There were no cases reported for polycythemia vera (PV) or lymphoma. (4) Sex distribution was nearly equivalent with the exception in patients with multiple sclerosis where there was female predominance (12 of 16 with reported data). (5) For pooled analysis, the average duration of treatment with IFN before TMA was diagnosed was 40.4 months. (6) Comparative analysis showed that patients with MS required the highest cumulative dose exposure before developing TMA (MS 68.6 months, CML 35.5 months, HCV 30.4 months). (7) Cases of confirmed TTP (where A disintegrin and Metalloprotease with thrombospondin type 1 motif 13: ADAMTS 13 level was measured) showed presence of an inhibitor. (8) In all cases of confirmed TTP, moderate to severe thrombocytopenia was a striking clinical feature at presentation while this was not a consistent finding in all other cases of TMA. (9) Outcome analysis revealed complete remission in 27 (40%), persistent chronic kidney disease (CKD) in 28 (42%) and fatality in 12 patients (18%). (10) Treatment with corticosteroids, plasma exchange and rituximab resulted in durable responses.
干扰素（IFN）与血栓性微血管病的发生有关，包括血栓性血小板减少性紫癜（TTP）和溶血尿毒综合征（HUS）。我们检索了从 1993 年首次报道的相关文献，至 2016 年 7 月共 68 例。对这些数据的分析显示：（1）诊断时的平均年龄为 47 岁（95%可信区间，44-50）。（2）大多数病例见于 IFN 用于治疗慢性髓性白血病（CML）、多发性硬化症（MS）、慢性丙型肝炎病毒感染（HCV），以及 1 例毛细胞白血病（HCL）和 Sezary 综合征。（3）没有用于真性红细胞增多症（PV）或淋巴瘤的病例报告。（4）除多发性硬化症患者外，男女比例相当，其中女性占优（16 例中有 12 例报告了数据）。（5）对于汇总分析，诊断 TMA 前 IFN 治疗的平均时间为 40.4 个月。（6）对比分析显示，MS 患者在发生 TMA 前需要最高的累积剂量暴露（MS 68.6 个月，CML 35.5 个月，HCV 30.4 个月）。（7）确诊 TTP （ADAMTS13 水平测量）病例显示存在抑制剂。（8）所有确诊 TTP 病例在发病时均有明显的中度至重度血小板减少症这一临床特征，而其他 TMA 病例则并非如此。（9）预后分析显示，27 例（40%）完全缓解，28 例（42%）持续慢性肾脏病（CKD），12 例（18%）死亡。（10）皮质类固醇、血浆置换和利妥昔单抗治疗可获得持久缓解。
