Hennewig U, Schulz A, Adams O, Friedrich W, Göbel U, Niehues T
Department of Pediatric Oncology, Hematology and Clinical Immunology, Heinrich-Heine University Düsseldorf.
Klin Padiatr. 2007 Nov-Dec;219(6):343-7. doi: 10.1055/s-2007-985877.
Severe combined immunodeficiency (SCID) is a heterogeneous disease consisting of several different subtypes. Most subtypes present during infancy and without treatment, infections usually lead to early death. Diagnosis of SCID can be difficult as new subtypes are expected to be discovered soon. Late diagnosis is associated with a poorer outcome. Infections like rotavirus enteritis cannot be cleared in children with SCID due to impaired immunity. The aim of our study was to identify clues in children with rotavirus enteritis that aid to diagnose SCID early.
Total white blood counts in a cohort of SCID patients with persistent rotavirus infection at diagnosis (n=18) were compared to total white blood counts in matched control patients without SCID but with rotavirus infection.
Relative and absolute lymphopenia and eosinophilia were more common in SCID patients (p<0.005).
In infants with rotavirus infection, a full blood count should be performed: Eosinophilia and/or lymphopenia raise a high suspicion of SCID.
重症联合免疫缺陷病（SCID）是一种由几种不同亚型组成的异质性疾病。大多数亚型在婴儿期出现，未经治疗的话，感染通常会导致早期死亡。由于预计很快会发现新的亚型，SCID的诊断可能会很困难。诊断延迟与较差的预后相关。由于免疫力受损，轮状病毒肠炎等感染在SCID患儿中无法清除。我们研究的目的是在轮状病毒肠炎患儿中寻找有助于早期诊断SCID的线索。
将一组诊断时患有持续性轮状病毒感染的SCID患者（n=18）的全血细胞计数与匹配的无SCID但患有轮状病毒感染的对照患者的全血细胞计数进行比较。
相对和绝对淋巴细胞减少及嗜酸性粒细胞增多在SCID患者中更为常见（p<0.005）。
对于感染轮状病毒的婴儿，应进行全血细胞计数：嗜酸性粒细胞增多和/或淋巴细胞减少高度怀疑患有SCID。
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