Lobut J B, Reinert P, Kohout G, Lemerle S, Bernaudin F, Cohen R
Centre Hospitalier Intercommunal, Créteil.
Ann Med Interne (Paris). 1987;138(6):416-8.
A 15 month old child with a history of multiple infectious diseases was admitted to hospital for investigation of pyrexia and general ill health. She was anaemic with a persistent neutropaenia associated with hypergamma globulinemia, indicating intense autoimmune activity: cellular immunity was abnormal with a normal total lymphocyte count but a very low T4/T8 ratio. HIV serology was positive; the virus was isolated from a lymph node biopsy specimen. The parents for the child were HIV positive and the father went on to develop full blown AIDS. The neutropaenia was constant over the two years of follow-up and granulo-immunofluorescence studies suggested an autoimmune origin. In contrast to autoimmune thrombocytopaenia and haemolytic anaemia, autoimmune neutropaenia is a rare condition. A few cases have been reported in adult AIDS. Our case is of additional interest as it illustrates the vertical mode of transmission of AIDS.
一名有多种传染病史的15个月大儿童因发热和全身健康状况不佳入院接受检查。她患有贫血,伴有持续性中性粒细胞减少症,并伴有高丙种球蛋白血症,提示存在强烈的自身免疫活动:细胞免疫异常,总淋巴细胞计数正常,但T4/T8比值极低。HIV血清学检查呈阳性;病毒从淋巴结活检标本中分离出来。患儿的父母HIV检测呈阳性,父亲随后发展为典型的艾滋病。在两年的随访中,中性粒细胞减少症持续存在,粒细胞免疫荧光研究提示为自身免疫性起源。与自身免疫性血小板减少症和溶血性贫血不同,自身免疫性中性粒细胞减少症是一种罕见的疾病。成人艾滋病中已有少数病例报道。我们的病例更具意义,因为它说明了艾滋病的垂直传播方式。