ORAL MANIFESTATIONS IN BLOOD
DYSCRASIAS
Many systemic hematological diseases have
cutaneous findings as well as oral mucosal findings that are unique and can be
the presenting clinical sign of the underlying disease. Awareness of the oral
manifestations of these disorders is of paramount importance. Oral
manifestations of blood dyscrasias can be seen in agranulocytosis, pernicious
anemia, leukemia, polycythemia vera, and thrombotic thrombocytopenic purpura
(TTP).
Clinical Findings: Agranulocytosis has been shown to produce oral ulcerations and erosions.
Many different causes of agranulocytosis may result in these clinical findings.
Medication-induced agranulocytosis is the most frequent cause of a decreased
absolute neutrophil count of less than 500/µL. Many medications can cause this
reaction, including dapsone, methotrexate, and a host of chemotherapeutic
agents. A rare autosomal recessively inherited disease called infantile genetic
agranulocytosis or Kostmann disease has been described. These patients present
in the first months of life with recurrent oral ulcerations, multiple bacterial
infections, and severely depressed absolute neutrophil counts. Death is the
norm by 1 year of age unless the disease is correctly diagnosed and treated. Successful
therapy is achieved with granulocyte colony-stimulating factor (G-CSF) or, in
more advanced cases, with bone marrow transplantation. Even with successful
G-CSF treatment, patients still develop oral ulcerations and severe periodontal
disease. This is caused by the lack of an antimicrobial peptide, which allows
certain bacteria to proliferate unabated. The species most frequently found is Actinobacillus
actinomycetes comitans.
Pernicious anemia is caused by a deficiency of vitamin
B12. This deficiency is most commonly seen in individuals with an inability to
absorb vitamin B12 or in strict vegetarians. Pernicious anemia can manifest
with a macrocytic anemia and neurological complications. Hunter’s glossitis is
a form of atrophic glossitis that affects the tongue, leading to atrophy of the
tongue filiform and fungiform papillae. The tongue takes on a beefy red
appearance with a smooth surface. Varying amounts of glossodynia and decreased
ability to taste are also found.
Gingival infiltration with leukemic cells may be the
presenting sign of an acute leukemia, and gingival bleeding is the most
frequent oral manifestation of leukemia. Oral ulcerations are commonly
associated with the gingival leukemic hypertrophy. The gums appear red and
swollen, with varying degrees of gingivitis. The gums may grossly enlarge to
cover the majority of the teeth. This form of leukemic infiltration is seen
almost exclusively in acute myelomonocytic leukemia (M4) and acute monocytic
leukemia (M5). It is estimated to occur in two thirds of patients with M5
disease and in 20% of those with M4 disease. Other forms of leukemia have been
implicated in causing gingival enlargement to a much lesser degree.
Polycythemia vera (previously termed polycythemia rubra vera) is caused by excessive production of red blood cells, which results in
abnormally high hemoglobin and hematocrit values. The majority of cases are
complicated by thrombosis. Most of these patients have a mutation in the JAK2
gene, which encodes a Janus family tyrosine kinase protein. The ability to
test for these mutations has made diagnosis much easier. Oral manifestations
are limited to the tongue and gingival mucosa. The tongue may become slightly enlarged, smooth, and hyperemic.
Bleeding from the gingival mucosa can also be seen. The disease is manifested
by many other systemic signs and symptoms.
TTP is a rare, life-threatening disease that can
develop rapidly. It is manifested by the formation of microthrombi throughout
the small vasculature. This causes multisystem organ failure and rapid death if
not promptly treated. Most cases have been found to be caused by a hereditary defect in the ADAMTS13 gene
or by decreased platelet levels caused by medications or autoimmunity. ADAMTS13
is a gene that encodes a plasma metalloprotease, which is important in
regulating von Willebrand factor function. Oral manifestations of the disease
include widespread petechiae and ecchymosis of the tongue, gingival, labial,
and buccal mucosa. Petechial hemorrhages of the gums may appear later in the course of the disease.
