Beatty P A, Stroncek D F
Sheboygan Clinic, WI.
J Lab Clin Med. 1992 Jun;119(6):718-23.
Autoimmune neutropenia is thought to be an uncommon disorder in adults. Over a 2-year period, however, autoimmune neutropenia was diagnosed in seven adults in a county with a population of approximately 105,000 people. The median age of the patients was 52 years old (range of 22 to 81 years), and five of the seven patients were women. All seven patients had at least one other symptom of autoimmune disease. Three patients had splenomegaly, three patients had positive direct antiglobulin tests, and two patients had immune thrombocytopenia. Antibodies reacting to neutrophils were detected by either granulocyte agglutination (GA) or granulocyte immunofluorescence (GIF) testing in five of the seven patients. Antibodies in four patients reacted with an 80 kd neutrophil membrane glycoprotein, and antibodies from two people reacted with a 60 kd membrane glycoprotein. Three patients were given treatment with splenectomy, which resulted in only transient improvement in the neutrophil counts. Serious infections occurred in only three patients over the 2 years of observation. In summary, autoimmune neutropenia in adults may occur more often than appreciated. Most cases of autoimmune neutropenia in adults appear to be associated with other autoimmune phenomena.
自身免疫性中性粒细胞减少症被认为在成人中是一种罕见的疾病。然而,在一个约有105,000人口的县,在两年时间里,有7名成人被诊断出患有自身免疫性中性粒细胞减少症。患者的中位年龄为52岁(范围为22至81岁),7名患者中有5名是女性。所有7名患者都至少有另一种自身免疫性疾病的症状。3名患者有脾肿大,3名患者直接抗球蛋白试验呈阳性,2名患者有免疫性血小板减少症。在7名患者中的5名中,通过粒细胞凝集(GA)或粒细胞免疫荧光(GIF)检测发现了与中性粒细胞反应的抗体。4名患者的抗体与一种80kd的中性粒细胞膜糖蛋白反应,2人的抗体与一种60kd的膜糖蛋白反应。3名患者接受了脾切除术治疗,这仅使中性粒细胞计数有短暂改善。在两年的观察期内,只有3名患者发生了严重感染。总之,成人自身免疫性中性粒细胞减少症的发生率可能比人们意识到的更高。成人自身免疫性中性粒细胞减少症的大多数病例似乎与其他自身免疫现象有关。