自身免疫性淋巴细胞增生综合征（ALPS）：免疫性血细胞减少的罕见病因。
Autoimmune lymphoproliferative syndrome (ALPS): a rare cause of immune cytopenia.
作者信息
John M Joseph, Rajasekhar Reena, Mathews Vikram
机构信息
Department of Hematology, Christian Medical College, Vellore, India.
出版信息
Indian Pediatr. 2008 Feb;45(2):148-50.
PMID:18310796
Abstract
Autoimmune Lymphoproliferative syndrome (ALPS) is an inherited disorder manifesting with autoimmune cytopenia, lymphadenopathy and splenomegaly. The differential diagnosis includes infections, autoimmune disorders or malignancies. The disease is characterized by accumulation of double negative (CD3+ CD4- CD8-) T cells (DNT) in the peripheral blood. We describe a case and review the literature.
摘要
自身免疫性淋巴细胞增生综合征（ALPS）是一种遗传性疾病，表现为自身免疫性血细胞减少、淋巴结病和脾肿大。鉴别诊断包括感染、自身免疫性疾病或恶性肿瘤。该疾病的特征是外周血中双阴性（CD3+ CD4- CD8-）T细胞（DNT）的积累。我们描述了一例病例并对文献进行了综述。
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