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[婴儿期的伊文氏综合征]

[Evans syndrome in infants].

作者信息

Flores-Montes Olivia Alejandra, Escobar-Orduño Martha Cecilia, Lozano-Garcidueñas Mónica, Valle-Leal Jaime Guadalupe

机构信息

Departamento de Pediatría, Hospital General Regional Número Uno, Instituto Mexicano del Seguro Social, Ciudad Obregón, Sonora, México.

Departamento de Hematología Pediátrica, Hospital General Regional Número 14, Instituto Mexicano del Seguro Social, Hermosillo, Sonora, México.

出版信息

Bol Med Hosp Infant Mex. 2017 Mar-Apr;74(2):141-146. doi: 10.1016/j.bmhimx.2017.01.004. Epub 2017 Mar 11.

Abstract

BACKGROUND

Evans syndrome is characterized by the reduction of at least two blood cell lineages in the absence of other diagnoses; it was previously described as the simultaneous or sequential development of autoimmune hemolytic anemia and immune thrombocytopenia with unknown etiology. An incidence of 37% and mortality rate of 10% were reported for Evans syndrome.

CLINICAL CASES

We report the clinical presentation and evolution of Evans syndrome in two infants who were initially diagnosed with immune thrombocytopenia. The clinical diagnosis was supported on complementary studies, where hematological disorders were corroborated. Both cases received treatment with steroids and intravenous immunoglobulin.

CONCLUSIONS

For the management of children with thrombocytopenia, the pediatrician must analyze for other cell lineage disorders. In the cases that we report here, we found the presence of autoimmune hemolytic anemia and monocytosis. Therefore, infectious and immunological studies must be included. The first-line treatment of choice are steroids, and intravenous immunoglobulin can be considered if severe immune thrombocytopenia is associated, as observed in these cases.

摘要

背景

伊文斯综合征的特征是在无其他诊断的情况下至少两种血细胞谱系减少;它曾被描述为病因不明的自身免疫性溶血性贫血和免疫性血小板减少症同时或相继发生。据报道,伊文斯综合征的发病率为37%,死亡率为10%。

临床病例

我们报告了两名最初被诊断为免疫性血小板减少症的婴儿的伊文斯综合征临床表现及病情发展。辅助检查支持临床诊断,证实了血液系统疾病。两例均接受了类固醇和静脉注射免疫球蛋白治疗。

结论

对于血小板减少症患儿的管理,儿科医生必须分析是否存在其他细胞谱系疾病。在我们报告的病例中,我们发现存在自身免疫性溶血性贫血和单核细胞增多症。因此,必须进行感染性和免疫性检查。首选的一线治疗是类固醇,如果伴有严重免疫性血小板减少症,如在这些病例中观察到的情况,可考虑静脉注射免疫球蛋白。

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