Leukocyte adhesion deficiency type II (LAD II) is a rare, autosomal, recessive inherited immunodeficiency disease that induces frequent and recurrent infections, persistent leukocytosis, severe mental and growth retardation, and impaired wound healing. The Bombay blood group is a rare blood group phenotype that is characterised by the deficiency of H, A, and B antigens on the surface of red cells. LAD II and the Bombay blood group are always seen together, because both of them are associated with a global defect in the common pathway of fucose metabolism. Here we report the case of an 11-year-old boy with LAD II, who presented with the Bombay blood group. Agglutination with strength of 4+ was detected in all cross-matching due to erythrocyte transfusions for our patient. Therefore, the Bombay blood group was incidentally determined due to deficient expression of the CD15 adhesion molecules on the surface of the leukocytes according to the results of flow cytometry. Upon detecting the Bombay blood type, LAD II was then diagnosed as a result of flow cytometry and the clinical findings of mental retardation and history of recurrent infections such as abscesses.

中文翻译：

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儿童白细胞黏附缺乏症II型和孟买血型的晚期诊断：罕见病例报告。

II型白细胞粘附缺乏症（LAD II）是一种罕见的常染色体隐性遗传性免疫缺陷病，可引起频繁和反复感染，持续性白细胞增多，严重的智力和生长迟缓以及伤口愈合不良。孟买血型是一种罕见的血型表型，其特征是红细胞表面缺乏H，A和B抗原。LAD II和孟买血型总是在一起出现，因为它们都与岩藻糖代谢共同途径中的整体缺陷有关。在这里，我们报告了一个11岁男孩LAD II的病例，该男孩出现在孟买血型中。由于我们患者的所有红细胞输注，在所有交叉匹配中均检测到凝集强度为4+。因此，根据流式细胞仪的结果，由于CD15粘附分子在白细胞表面的表达不足，偶然地确定了孟买血型。一旦检测到孟买的血型，就可以通过流式细胞仪和智力低下的临床发现以及复发性感染（如脓肿）的历史诊断LAD II。