PubMed1993
A leukocyte adhesion deficiency characterized by recurrent (predominantly bacterial) infections, lack of extravascular polymorphonuclear leukocyte (PMN) and pus formation has been described first in humans and then in dogs, and recently also in cattle. Because of important clinical similarities, a unitary explanation for the leukocyte adhesion deficiency (LAD) syndrome in mammals is proposed, inasmuch that an intrinsic leukocyte defect (i.e. mutations in genes encoding the common CD18 subunit), is thought to cause the disease. However, thus far, the hallmark of such intrinsic leukocyte defects, notably their heritability (or familial incidence), has not (yet) been unequivocally demonstrated. This is the first report to describe the occurrence of four Dutch bovine LAD (BLAD) cases with the clearest familial clustering observed to date. The diagnosis was based on the clinical features of very poor thriving, in general, of the calves, hyperneutrocytosis without appreciable left shift, and the absence of PMN CD11a, or CD11b, or CD11c using monoclonal antibodies (mAb) and/or Concanavalin A binding activity of PMN lysates in immunoblots. Interestingly, a familial clustering was observed also for below-normal PMN CD11c expression. Thus, a cow with low CD11c expression (50.4%) and delivering three of the study BLAD calves, also had a healthy descendant with low (44.9%) PMN CD11c expression. These findings suggested the possibility that both subnormal expression and lack of PMN CD11 expression are inheritable factors in cattle. Furthermore, a large prospective study using the present mAb for selecting relatives expressing the complete spectrum (0 to > or = 90%) of PMN CD11/CD18 expression would create a comprehensive study population for understanding both the role of genetic factors and of survival strategies in BLAD.