Birat Medical College and Teaching Hospital, Biratnagar, Morang, Nepal.
Department of Community Medicine, Birat Medical College and Teaching Hospital, Biratnagar, Morang, Nepal.
JNMA J Nepal Med Assoc. 2022 May 5;60(249):482-484. doi: 10.31729/jnma.7421.
Evans syndrome is defined as the concomitant or sequential association of warm autoimmune hemolytic anaemia with immune thrombocytopenia, and less frequently autoimmune neutropenia. It is associated with non-cross-reacting auto-antibodies directed against antigens specific to red blood cells, platelets or neutrophils. Clinical symptoms could be related to hemolysis and thrombocytopenia. Evans syndrome is a rare diagnosis of exclusion. The first-line treatment of Evans syndrome is intravenous corticosteroids or intravenous immunoglobulins and second-line treatment with rituximab or splenectomy for those who are refractory to steroids. Here is a case of a fifty-year-old- female who presented with bleeding from the mouth and gums, bluish patches over the shin and trunk along with generalised weakness and severe backache. We are interested in reporting this case because the presentation of patients with such scenarios on our part will compel the treating physician to overlook Evans syndrome and get it underdiagnosed.
case reports; immunoglobulins; neutropenia; thrombocytopenia.
中文译文:
Evans 综合征的定义为温抗体自身免疫性溶血性贫血与免疫性血小板减少症同时或相继发生,且较少见的是自身免疫性中性粒细胞减少症。它与针对红细胞、血小板或中性粒细胞特异性抗原的非交叉反应性自身抗体有关。临床症状可能与溶血和血小板减少症有关。Evans 综合征是一种罕见的排除性诊断。Evans 综合征的一线治疗是静脉内皮质类固醇或静脉内免疫球蛋白,对于对类固醇耐药的患者,二线治疗是利妥昔单抗或脾切除术。这里报告了一例五十岁女性患者,她出现口腔和牙龈出血、小腿和躯干出现蓝色斑块,伴有全身无力和严重背痛。我们有兴趣报告这个病例,因为我们的患者出现这种情况会使治疗医生忽视 Evans 综合征并导致其漏诊。
关键词:病例报告;免疫球蛋白;中性粒细胞减少症;血小板减少症