Neutrophil Migration

Clinical Features

In 3 children with congenital ichthyosis and recurrent infections with Trichophyton rubrum, Miller et al. (1973) identified an abnormality of neutrophil movement. Numbers, morphology, and phagocytic and bactericidal activities were normal. Although random mobility was normal, chemotaxis of leukocytes from the patients and their fathers was deficient. (In the 'lazy leukocyte syndrome' (150550), random movement is also defective.) A girl in one family and a brother and sister in a second were affected. In each family the father showed the same defect of neutrophil movement. The father of the 2 affected sibs had been plagued by recurrent Trichophyton rubrum infections but did not have ichthyosis.

Jacobs and Norman (1977) found a cellular chemotactic defect in the neutrophils of both parents and 3 of 4 children who suffered from unusually severe bacterial eczema, asthma and recurrent bacterial skin infections, all starting in the first month of life. All affected members had HLA-B12; the normal child did not. The 2 most severely affected children were homozygous for HLA-B12.

Mapping

Ruutu et al. (1977) found an association between monosomy 7 (252270) and defective chemotaxis, suggesting that a gene for normal chemotactic or chemokinetic response of neutrophils may be located on that chromosome.

De la Chapelle et al. (1982) showed that the locomotion defect of granulocytes in monosomy 7 involves random locomotion, chemotaxis, and chemokinesis.