Marsh W L, Oyen R, Nichols M E
Vox Sang. 1976 Nov;31(5):356-62. doi: 10.1111/j.1423-0410.1976.tb04659.x.
Leukocytes of nine unrelated boys with X-linked chronic granulomatous disease lack Kx antigen. In three of these cases, the red cells also lack Kx and have the McLeod phenotype and abnormal morphology. X-linked chronic granulomatous disease CGD can thus be separated into two types. Type I cases have an antigenic deficiency that is restricted to the phagocytic leukocytes while in type II, the deficiency involves both leukocytes and red cells. Red cells of type II CGD patients have enhanced i antigen activity suggesting that they are under hemopoietic stress. Normal Kx synthesis is directed by an X-linked gene named X1k. Three variants, X2k, X3k, and X4k order the different permutations of leukocyte and red cell Kx antigen production that have been recognized.
9名患有X连锁慢性肉芽肿病的无关男孩的白细胞缺乏Kx抗原。在其中3例中，红细胞也缺乏Kx，具有麦克劳德表型和异常形态。因此，X连锁慢性肉芽肿病（CGD）可分为两种类型。I型病例存在仅限于吞噬性白细胞的抗原缺陷，而II型病例的缺陷则涉及白细胞和红细胞。II型CGD患者的红细胞具有增强的i抗原活性，表明它们处于造血应激状态。正常的Kx合成由一个名为X1k的X连锁基因指导。X2k、X3k和X4k这三个变体排列了已识别的白细胞和红细胞Kx抗原产生的不同组合。
