Lazy Leukocyte Syndrome—an Enigma Finally Solved?

Excerpt

The initial report in the Lancet [1] described two unrelated children, a 5-year-old girl and a 2-year-old boy, with a history of early onset gingivitis, stomatitis, and recurrent otitis media. The family histories were unremarkable. Both children had severe neutropenia with normal bone marrow examination. Immunological parameters, including serum immunoglobulin levels and lymphocyte responses to phytohemagglutinin, were normal. While phagocytosis and bactericidal activity were similar to controls, the migration of the patients' neutrophils was markedly defective, both in vivo (by Rebuck skin window) and in vitro (using a modified Boyden chamber). The fact that both random migration and chemotaxis were defective pointed to an intrinsic leukocyte defect and raised, in the authors' opinion, the possibility of a membrane defect in patient neutrophils [1]. …