11.12.2 Neutropenias
The neutropenias comprise a heterogeneous group of blood disorders that are
characterized by a periodic or persistent reduction in the number of circulating
polymorphonuclear neutrophils. Neutropenias can be drug-induced or be secondary to
severe bacterial or viral infections or autoimmune diseases such as lupus
erythematosus. Cyclic neutropenia, benign familial neutropenias, and severe familial
neutropenias are all heritable conditions transmitted as autosomal-dominant traits and
diagnoses are often made during early childhood. The chronic benign neutropenia of
childhood is diagnosed between 6 and 24 months of age and is characterized by
frequent and multiple pyogenic infections of the skin and mucous membranes.
The periodontal problems associated with the neutropenias are very similar, and in
many cases the patient presents with a localized or generalized aggressive
periodontitis. Occasionally, the primary dentition may not be involved, and clinical
signs do not appear until the permanent dentition has erupted. The gingiva are
inflamed and oedematous; gingival recession, ulceration, and desquamation can also
occur.
The treatment of a neutropenic-induced periodontitis involves local removal of plaque
and calculus. Strict plaque control measures are difficult to achieve in younger
children, so use of an antibacterial mouthrinse may prove useful.
11.12.3 Chediak-Higashi syndrome
This is a rare and very often fatal disease inherited as an autosomal-recessive trait.
Clinical features include partial albinism, photophobia, and nystagmus. The patients
suffer from recurrent pyogenic infections and malignant lymphoma⎯which is
accompanied by neutropenia, anaemia, and a thrombocytopenia. The neutrophils
show defects in migration, chemotaxis, and phagocytosis producing a diminished
bactericidal capacity.
Periodontal changes associated with the syndrome include severe gingival
inflammation and rapid, and extensive, alveolar bone resorption that can lead to
premature exfoliation. The nature of the changes has not been fully established, but
they may be plaque-induced, secondary to infection, or related to the underlying
defect in neutrophil function.
11.12.4 Leucocyte-adhesion deficiency syndrome (LAD)
This autosomal-recessive trait is characterized clinically by a delayed separation of
the umbilical cord, severe recurrent bacterial infections, impaired wound healing,
formation of pus, and an aggressive gingivitis, which may be the presenting sign of
the disorder. Consanguinity between the parents of affected children confirms the
mode of the inheritance as autosomal-recessive.
The syndrome demonstrates the important role of leucocytes (and other white blood
cells) in protecting the host against periodontal disease. Moderate pheno- types,
however, may appear relatively disease-free, but then develop symptoms and progress