Defective neutrophil function in myotonic dystrophy

Abstract

Polymorphonuclear leukocytes (PMNs), obtained from 10 patients with myotonic dystrophy and 39 age-matched controls, were tested for chemotactic activity, chemiluminescence (CL), and phagocytosis. PMNs from 8 of the 10 patients had depressed chemotactic indexes (30 +/- 23) as compared to controls (61 +/- 18) (P less than 0.0003), and 6 of the 10 patients' PMNs produced lower peaks in CL (less than 90%) than those of controls. Phagocytosis and bactericidal activity were evaluated in 7 patients and were normal. Control PMNs incubated in patient serum produced normal chemotactic activity and CL. These results indicate defects in 2 PMN membrane-associated activities in myotonic dystrophy and suggest that the PMN is affected by the generalized membrane abnormality underlying myotonic dystrophy.