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Hematologic Condition Associated

with Periodontal Disease in Children

Dian Puspita Sari

Soft tissue lesions of the oral cavity
are common in children
Distinguishing between findings that
are normal and those that are
indicative of gingivitis, periodontal
disease, local or systemic infection,
and potentially lifethreatening
systemic conditions is important

The loss of periodontal attachment in

children, manifest by tooth mobility
or premature loss, can be a symptom
of neoplasia, immunodeficiency, or
metabolic defects
The early detection and treatment of
these conditions can be life-saving.

A rare disorder
Histiocytic infiltration of the bones,
skin, liver, or other organs
Langerhans cell histiocytosis (LCH)
presents with single or multiple-site
The skin, oral mucosa, bone, and
lymph nodes are typical locations for
single-site involvement

Multisite involvement occurs in the

liver, spleen, lungs, bone marrow,
and gastrointestinal and central
nervous systems
Between 10 and 20 percent of
patients present with infiltration of
the oral cavity, usually the posterior

The typical dental presentation of

LCH is eruption of the primary molars
at or soon after birth

Additional oral manifestations include

pain; ulceration; enlargement,
inflammation, or recession of the
gingiva; and mobility of teeth
because of expansion of the alveolar
Dental radiographs may show
discreet, destructive bone lesions
that make the teeth appear to be
"floating on air"

Periosteal new bone formation and

slight root resorption also may be
Cases may present as aggressive
periodontitis lesions that do not
respond to routine periodontal
therapy, despite the presence of
periodontal flora typically associated
with periodontitis

When periodontal involvement is

suspected to be a manifestation of
LCH, biopsy of the gingiva or
periodontal tissues is needed
The diagnostic evaluation for children
in whom a diagnosis of LCH is being
considered is extensive and should
be performed in consultation with a
pediatric hematologist

Leukemia, particularly the monocytic
type, can cause gingival enlargement
because of infiltration of the gingival
Leukemic gingival enlargement is
typically painless, shiny, red, and
Bleeding is common and can make it
difficult to maintain oral hygiene

Necrotic ulceration and involvement

of the underlying bone also can occur
The inflammation that results may
act as a stimulus for further gingival
Additional symptoms include fever,
malaise, easy bruising or bleeding,
and bone or joint pain.

The diagnosis of leukemia should be

considered in patients who have
hemorrhagic gingival edema and
anemia, thrombocytopenia, or
abnormal leukocyte and differential
counts on complete blood count

The treatment of leukemia (both

lymphocytic and myelogenous
leukemia) can have oral complications,
- Mucositis
- Oral infection with candida, herpes
virus, or other opportunistic organisms
- Gingival inflammation

- Spontaneous gingival bleeding

(because of thrombocytopenia)
- Gingival squamous cell carcinoma
(as a complication of graft versus
host disease in bone marrow
transplant recipients)

The chemotherapy-induced oral

complications in patients with
leukemia are more prevalent
immediately after administration of

The oral complications of

chemotherapy can be diminished by
aggressive preventive care
Comprehensive oral examination
before the initiation of cancer
The oral cavity is a reservoir for
many microorganisms with the
potential to cause systemic infection
in the immunocompromised host

In addition, dental plaque causes gingivitis and

gingival bleeding
Careful brushing with a soft toothbrush should
be continued throughout therapy
Although it has been recommended that
toothbrushing be suspended or replaced by
cleaning with sponge-tipped brushes ("toothettes")
when platelet counts are low, these approaches
are not adequate for plaque removal, and available
evidence suggests problems are more likely to
arise when oral hygiene is poor

Neutropenia is a hematologic
disorder characterized by reduced
numbers of circulating neutrophils.
It is diagnosed when the absolute
neutrophil count (ANC) is less than

Neutrophils are an important

component of the host response to
pathogenic dental plaque in the
gingival sulcus, and patients with
neutropenia are at risk for severe
gingivitis and pronounced alveolar
bone loss.

Periodontal disease occurs in the

following types of childhood
Congenital neutropenia
Autoimmune neutropenia
Cyclic neutropenia

Congenital neutropenia occurs in
several conditions where there is a
marked decrease in (or lack of)
circulating neutrophils from the time
of birth
an estimated frequency of two cases
per million population

Children with congenital neutropenia

are susceptible to recurrent infection,
often due to staphylococci and
Oral lesions, otitis media, respiratory
infection, cellulitis, and skin
abscesses are the most common.
These infections heal slowly and may
be fatal

Oral manifestations of congenital

neutropenia include ulcers, severe
gingivitis, alveolar bone loss, gingival
recession, tooth mobility, and
premature tooth exfoliation.
The treatment of congenital
neutropenia usually involves the
administration of granulocyte colonystimulating factor (G-CSF)

Autoimmune neutropenia (AIN) is caused
by granulocyte-specific antibodies.
AIN has been associated with a variety of
underlying diseases, including viral
infection, collagen vascular disease,
primary abnormalities of B or T
lymphocytes or natural killer (NK) cells,
idiopathic thrombocytopenic purpura
(ITP), and autoimmune hemolytic anemia

Benign neutropenia of infancy and

childhood typically occurs in infants
between the ages of 5 to 15 months,
but the range extends from one
month to adulthood
Severe gingivitis and periodontal
disease may result without
preventive measures.

Cyclic neutropenia
Cyclic neutropenia is characterized
by regular oscillations in the numbers
of circulating neutrophils, monocytes,
eosinophils, lymphocytes, and
The cycles typically occur at 21-day
intervals, but the intervals can range
from 15 to 35 days

Cyclic neutropenia can have onset in

childhood or adulthood
Childhood onset is more common
and appears to be a genetic
condition with autosomal dominant

Dental management
Early dental referral and highly
motivated parents are the keys to
successful dental management of
children with neutropenia
Neutropenia predisposes the child to
hemorrhagic gingivitis and periodontal
disease, but the progression of bone
loss is because of the host response to
pathogenic subgingival plaque

Thus, scrupulous oral hygiene,

antimicrobial rinses, frequent
professional tooth cleaning, and
targeted antibiotic therapy can delay
or halt periodontal bone loss.
Antibiotic therapy for neutropenic
children with periodontal disease is
determined by microbial cultures of
the gingival sulcus

The organisms most commonly cultured in

children with periodontal disease include
Prevotella intermedia, Actinobacillus
actinomycetemcomitans, Eikenella corrodens,
and Capnocytophaga sputigena
Eradication and control of these pathogens is
essential in the treatment of periodontal
Periodic surveillance cultures will help to
determine the need for repetition of antibiotic

The treatment of neutropenic

children with periodontal disease is
usually more successful in children
with localized than with generalized
periodontal disease; generalized
periodontal disease may be
refractory to antibiotic therapy
without correction of the underlying
neutrophil defect.

Treatment of the underlying disorder

with administration of G-CSF, and
normalization of neutrophil counts,
may not be sufficient to maintain oral
health because of possible
associated functional neutrophil
defects; this underscores the
significance of professional dental
care for such patients