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儿童血小板减少症与人类免疫缺陷病毒

Thrombocytopenia and human immunodeficiency virus in children.

作者信息

Ellaurie M, Burns E R, Bernstein L J, Shah K, Rubinstein A

机构信息

Department of Pediatrics, Albert Einstein College of Medicine, Bronx, New York 10461.

出版信息

Pediatrics. 1988 Dec;82(6):905-8.

PMID:3186382
Abstract

Thrombocytopenia occurs in 13% of children with symptomatic human immunodeficiency virus (HIV) infection. The clinical and laboratory course of 19 children infected with HIV with thrombocytopenia is described. Bone marrow aspirates showed normal to increased numbers of megakaryocytes. Levels of antiplatelet antibodies were increased in 80% of the children and circulating immune complexes were found in 74%. Clinically significant hemorrhage leading to anemia occurred in five patients, and CNS bleeding led to a fatal outcome in an additional three children. Spontaneous remission of thrombocytopenia occurred in three of the 19 subjects. High-dose IV gamma-globulin was effective in increasing the platelet counts of six of 15 patients (40%) but resulted in a sustained remission in only one subject. Oral prednisone was effective in increasing the platelet count of two thirds of those whose platelet counts could not be controlled by IV gamma-globulin. Bleeding manifestations were eliminated in all patients whose platelet counts increased significantly. Of the 11 children whose counts increased either spontaneously or as a result of therapy, eight remain alive (72%). In contrast, all of the eight patients whose platelet counts did not improve have died. Thrombocytopenia in children with HIV disease is engendered by immune mechanisms and is a major cause of morbidity and mortality. High-dose IV gamma-globulin and/or corticosteroids are temporarily effective in increasing the platelet count and reducing bleeding in about half of thrombocytopenic patients and are recommended for use. The ability to respond to therapy correlates with improved survival.

摘要

13%出现症状的人类免疫缺陷病毒(HIV)感染儿童会发生血小板减少症。本文描述了19例感染HIV且患有血小板减少症儿童的临床及实验室病程。骨髓穿刺显示巨核细胞数量正常或增多。80%的儿童抗血小板抗体水平升高,74%的儿童发现循环免疫复合物。5例患者发生导致贫血的具有临床意义的出血,另有3名儿童因中枢神经系统出血导致死亡。19名受试者中有3名血小板减少症自发缓解。大剂量静脉注射丙种球蛋白使15例患者中的6例(40%)血小板计数升高,但仅1例实现持续缓解。口服泼尼松对三分之二血小板计数无法通过静脉注射丙种球蛋白控制的患者有效,可使血小板计数升高。血小板计数显著升高的所有患者出血表现均消失。11例血小板计数自发升高或因治疗升高的儿童中,8例存活(72%)。相比之下,血小板计数未改善的8例患者均已死亡。HIV疾病患儿的血小板减少症由免疫机制引起,是发病和死亡的主要原因。大剂量静脉注射丙种球蛋白和/或皮质类固醇对约一半血小板减少症患者暂时有效,可升高血小板计数并减少出血,建议使用。对治疗的反应能力与生存率提高相关。

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