Alonso Hernandez-Company, Manuel Anguiano-Alvarez Victor, Amir Carmona Gonzalez Carlos, Sergio Rodriguez-Rodriguez, Allan Pomerantz, Xavier Lopez-Karpovitch, Juventina Tuna-Aguilar Elena
Hematology and Oncology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.
Comprehensive Cancer Center, Médica Sur Clinic and Foundation, Mexico City, Mexico.
Blood Res. 2017 Mar;52(1):44-49. doi: 10.5045/br.2017.52.1.44. Epub 2017 Mar 27.
Autoimmune hemolytic anemia (AIHA) is characterized by an autoimmune-mediated destruction of red blood cells. Warm AIHA (wAIHA) represents 60% of AIHA cases and is associated with the positive detection of IgG and C3d in the direct antiglobulin test (DAT). This study aimed to assess the clinical and laboratorial differences between primary and secondary wAIHA patients from a referral center in Mexico City.
All patients diagnosed with wAIHA in our institution from January 1992 to December 2015 were included and received corticosteroids as the first-line treatment. We analyzed the response to the first-line treatment, relapse-free survival, and time to splenectomy.
Eighty-nine patients were included. Secondary wAIHA represented 55.1% of the cases. At diagnosis, secondary wAIHA patients showed a DAT mixed pattern more frequently than primary wAIHA patients (36.7 vs. 17.5%, <0.001). In the survival analysis, patients with secondary wAIHA had a lower time to response (18 vs. 37 days, =0.05), median disease-free survival (28.51 vs. 50.95 weeks, =0.018), and time to splenectomy (43.5 vs. 61 wks, =0.029) than those with primary wAIHA. Due to economic constraints, rituximab was considered as the third-line treatment in only two patients.
Secondary wAIHA may benefit from a longer low-dose steroid maintenance period mainly due to its shorter time to relapse and time to splenectomy than primary wAIHA.
自身免疫性溶血性贫血(AIHA)的特征是自身免疫介导的红细胞破坏。温抗体型自身免疫性溶血性贫血(wAIHA)占AIHA病例的60%,与直接抗球蛋白试验(DAT)中IgG和C3d的阳性检测相关。本研究旨在评估墨西哥城一家转诊中心原发性和继发性wAIHA患者的临床和实验室差异。
纳入1992年1月至2015年12月在我们机构诊断为wAIHA的所有患者,并接受皮质类固醇作为一线治疗。我们分析了对一线治疗的反应、无复发生存率和脾切除时间。
纳入89例患者。继发性wAIHA占病例的55.1%。在诊断时,继发性wAIHA患者比原发性wAIHA患者更频繁地表现出DAT混合模式(36.7%对17.5%,<0.001)。在生存分析中,继发性wAIHA患者的反应时间(18天对37天,=0.05)、无病生存期中位数(28.51周对50.95周,=0.018)和脾切除时间(43.5周对61周,=0.029)均低于原发性wAIHA患者。由于经济限制,仅两名患者将利妥昔单抗视为三线治疗。
继发性wAIHA可能受益于更长时间的低剂量类固醇维持期,主要是因为其复发时间和脾切除时间比原发性wAIHA短。