Sugita K, Owada Y, Ozawa T, Sakakibara H, Eguchi M, Furukawa T, Saitoh K
Second Department of Pediatrics, Dokkyo University School of Medicine, Tochigi, Japan.
Int J Hematol. 1993 Jan;57(1):27-30.
We report an infant with autoimmune neutropenia (AIN), idiopathic thrombocytopenia (ITP), and IgG2/IgA deficiency. The patient was referred to our hospital at 5 months of age because of epistaxis and generalized petechiae. Physical examination revealed moderate hepatosplenomegaly. A complete blood count revealed a platelet count of 2.0 x 10(3) cells/microliters, and a white cell count of 3,600 cells/microliters, with severe neutropenia (less than 1% bands and segmented cells). Neutrophils and platelets adhering to megakaryocytes were decreased in the bone marrow. Tests for serum neutrophil-binding IgG (NB-IgG) and platelet-associated IgG (PA-IgG) were positive. A diagnosis of both AIN and ITP was made and therapy with intact-type gamma-globulin and prednisolone was initiated. Improvement occurred, but was temporary. A lack of serum IgA and IgG2 was noted during the clinical course. The patient has not been susceptible to bacterial infections but has had a severe clinical course with rubella and chickenpox.
我们报告了一名患有自身免疫性中性粒细胞减少症（AIN）、特发性血小板减少性紫癜（ITP）以及IgG2/IgA缺乏症的婴儿。该患者因鼻出血和全身性瘀点于5月龄时转诊至我院。体格检查发现有中度肝脾肿大。全血细胞计数显示血小板计数为2.0×10³个/微升，白细胞计数为3600个/微升，伴有严重的中性粒细胞减少症（带状核细胞和分叶核细胞少于1%）。骨髓中黏附于巨核细胞的中性粒细胞和血小板减少。血清中性粒细胞结合IgG（NB-IgG）和血小板相关IgG（PA-IgG）检测呈阳性。诊断为AIN和ITP，并开始用完整型γ-球蛋白和泼尼松龙进行治疗。病情有改善，但为暂时性。在临床病程中发现血清IgA和IgG2缺乏。该患者虽未易患细菌感染，但患风疹和水痘时临床过程严重。
