Infantile Genetic Agranulocytosis (Kostmann Type). A Case Report

Abstract

Severe periodontitis in a patient with infantile genetic agranulocytosis (Kostmann syndrome) is presented. This rare syndrome is inherited as an autosomal recessive pattern and characterized by severe neutropenia. The periodontal status and treatment of the patient is described. It is concluded that periodontal therapy including scaling, root planning, soft tissue curettage, and the use of selected antimicrobial agents can be successful in this particular syndrome.

Aggressive forms of periodontitis secondary to systemic disorders

“…A case report implicates herpes viruses in the pathogenesis of the periodontal defects associated with the condition (112). It has been suggested that a therapeutic regimen consisting of scaling and root planing, soft‐tissue curettage and the use of selected antimicrobial agents could be successful in the resolution of periodontal infection in subjects inflicted with this syndrome (77).…”

The genetic basis of periodontitis

“…A further neutrophil deficiency is found in infantile genetic agranulocytosis, a rare autosomal recessive disease where polymorphonuclear leukocyte numbers are very low and which has been associated with aggressive periodontitis (144).…”

How has neutrophil research improved our understanding of periodontal pathogenesis?

“…The association of severe periodontitis with a decrease in the number of circulating neutrophils is well documented in the literature. Situations such as neutropenia, agranulocytosis (Hou & Tsai 1988, Lamster et al 1987, Saglam et al 1995, Zubery et al 1991, Watanabe 1990, cyclic neutropenia (Rylander & Ericsson 1981, Scully et al 1982, Prichard et al 1984, Baer & Iacono 1994, Pernu et al 1996, da Fonseca & Fontes 2000, chronic benign neutropenia (Reichart & Dornow 1978), chronic idiopathic neutropenia (Baehni et al 1983, Kamma et al 1998, and familial benign chronic neutropenia (Deasy et al 1980, Stabholz et al 1990, Kirstila et al 1993, Porter et al 1994, are all asso-ciated with periodontitis. In neutropenic patients, the number of neutrophils sometimes can be restored with external administration of haematopoietic colony-stimulating factors, such as granulocyte colony-stimulating factor.…”