Background: Congenital neutropenia is characterized by an almost total absence of neutrophils and increased susceptibility to infection. Oral manifestations include ulcerations of mucous membranes, acute gingival inflammation with focal necrosis, and rapid loss of attachment. Treatment with recombinant human granulocyte colony-stimulating factor (rhG-CSF) increases neutrophil numbers and ameliorates the periodontal status. Methods: We report the treatment of a 22-year-old male with congenital neutropenia (Kostman syndrome), referred to us due to periodontal disease, and the effect of treatment on peripheral neutropenia. Diagnosis of neutropenia was made at year 1; at age 15, the patient started to receive injections of rhG-CSF, reducing the occurrence of infection and improving neutrophil count, although levels remained below normal. The patient underwent extraction of a molar at age 8; scaling, root planing, and modified Widman flaps at age 9; and oral hygiene maintenance every 2 to 3 months from age 18 to 21. At age 23, he initiated treatment at our periodontal clinic. The patient's gingiva was severely inflamed, and the dentition was covered with plaque and calculus. Attachment loss was advanced, all teeth were mobile, and bone loss was approximately 75% in most sites. Neutrophil counts were below normal, but other hematologic parameters were normal. Scaling and root planing were performed and the patient received antibiotics and chlorhexidine rinses twice each day for 2 weeks. Extracoronal splinting was performed, fluoride varnish was used to desensitize cervical areas, and tooth FDI #46 was restored. Root planing and deplaquing were repeated, and the patient received subgingival chlorhexidine irrigation 13 times over one year. Assessments were made on presentation, after the initial treatment, and at 1 and 2 years post-treatment. Results: Mean probing depth was reduced post-treatment with a further reduction during the maintenance period. This was correlated with an increase in attachment levels. Total white blood cells increased, due in part to an increase in neutrophils, reaching normal levels. Conclusions: This report demonstrates for the first time that periodontal therapy, resulting in decreased bacterial load, may result in restoration of normal levels of circulating neutrophils in individuals with congenital neutropenia under treatment with rhG-CSF. The results also suggest that periodontal pathogens may be associated with depressed neutrophil levels, even when patients receive treatment for neutropenia.
- Granulocyte colony-stimulating factor, recombinant
- Periodontal diseases/pathogenesis
- Periodontal diseases/therapy
ASJC Scopus subject areas