[自身免疫性溶血性贫血。43例病例回顾]

[Autoimmune hemolytic anemia. Review of 43 cases].

作者信息

López-Vidal Hernán, Peña Camila, Gajardo Claudia, Valladares Ximena, Cabrera C María Elena

机构信息

Universidad de Chile, Santiago, Chile.

Unidad de Hematología Clínica, Sección de Hematología, Hospital del Salvador, Santiago, Chile.

出版信息

Rev Med Chil. 2019 Jul;147(7):836-841. doi: 10.4067/S0034-98872019000700836.

DOI:10.4067/S0034-98872019000700836

PMID:31859981

Abstract

BACKGROUND

Autoimmune hemolytic anemia (AIHA) is an uncommon disease. In its presentation, it can be severe and even lethal. There is only one clinical report concerning this pathology in Chile.

OBJECTIVE

To describe the clinical characteristics and evolution of adult AIHA inpatients.

MATERIALS AND METHODS

Retrospective review of clinical records of adult AIHA inpatients between January 2010 and June 2018 was done. Demographic, clinical, laboratory and therapeutic information was analyzed. A descriptive, analytical and survival analysis was performed.

RESULTS

Forty-three adult patients diagnosed with AHIA were hospitalized in a period of 8 years. Median age was 63 years (range 22-86 years), mostly women (72%). Warm antibodies were detected in 36 cases (84%) and cold antibodies in seven. Seventy two percent of the patients had an underlying cause, and 58% were secondary to lymphoproliferative neoplasms. All patients except two, received steroids as initial treatment, with response in 37 (90%) of them. Three refractory patients received rituximab, with response in all of them, and relapse in one. Median follow-up was 38 months (range 2-98 months). Five year overall survival was 72%.

CONCLUSION

AHIA in adults inpatients is a heterogeneous disease, mainly due to warm antibodies, and to secondary etiology.

摘要

背景

自身免疫性溶血性贫血（AIHA）是一种罕见疾病。其临床表现可能较为严重，甚至危及生命。智利仅有一篇关于该病症的临床报告。

目的

描述成年AIHA住院患者的临床特征及病情发展。

材料与方法

对2010年1月至2018年6月期间成年AIHA住院患者的临床记录进行回顾性分析。分析人口统计学、临床、实验室及治疗信息，并进行描述性、分析性及生存分析。

结果

8年间有43例成年患者被诊断为AHIA并住院治疗。中位年龄为63岁（范围22 - 86岁），女性居多（72%）。36例（84%）检测到温抗体，7例检测到冷抗体。72%的患者有潜在病因，58%继发于淋巴增殖性肿瘤。除2例患者外，所有患者均接受了类固醇作为初始治疗，其中37例（90%）有反应。3例难治性患者接受了利妥昔单抗治疗，均有反应，1例复发。中位随访时间为38个月（范围2 - 98个月）。五年总生存率为72%。