SHELDON M. WOLFF, M.D., F.A.C.P.; DAVID C. DALE, M.D.; ROBERT A. CLARK, M.D.; RICHARD K. ROOT, M.D.; HARRY R. KIMBALL, M.D., F.A.C.P.
A striking clinical feature of the Chediak-Higashi syndrome (CHS) is a markedly increased susceptibility to pyogenic infections. Recent investigations have documented multiple defects in host defenses in patients with the syndrome. Neutropenia, a characteristic finding, is most likely related to intramedullary destruction of granulocytes. Defective leukocyte migration has been found in vivo by the skin window technique, and a cellular defect in Chemotaxis has been documented in vitro. Although phagocytosis is quantitatively normal, there is a failure in postphagocytic degranulation, as well as diminished intracellular bactericidal capacity, findings that may be related to ineffective delivery of lysosomal contents to the phagocytic vacuole. An abnormal distribution and reduced quantities of lysosomal enzymes have been shown. Some or all of these deficiencies in leukocyte regulation, structure, and function may contribute to the increased susceptibility to bacterial infections.
WOLFF SM, DALE DC, CLARK RA, ROOT RK, KIMBALL HR. The Chediak-Higashi Syndrome: Studies of Host Defenses. Ann Intern Med. ;76:293–306. doi: 10.7326/0003-4819-76-2-293
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Published: Ann Intern Med. 1972;76(2):293-306.
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