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Agranulocytosis

Definition

Agranulocytosis is a hematologic disorder characterized by a severe reduction or absence of granulocytes, in particular, the neutrophils (<500/mm³).

Etiology

Agranulocytosis may be caused by drug treatment and infections, although some cases are idiopathic.

Main Clinical Features

Oral lesions mainly occur early in the disease and are common.

Oral Manifestations

•Necrotic ulcers covered by a gray-white pseudomembrane without a red halo

•Increased salivation and painful mastication are common symptoms

•Severe necrotizing gingivitis with destruction of periodontal tissues may occur

Systemic Manifestations

•Sudden chills, fever, malaise, and sore throat

•Within 12-24 hours, signs and symptoms of respiratory, and/or gastrointestinal, or other bacterial infections often develop

•Risk of infections is usually related to the degree of neutropenia

Diagnosis

The clinical diagnosis should be confirmed by a complete blood count and bone marrow aspiration.

Differential Diagnosis

•Neutropenia

•Cyclic neutropenia

•Aplastic anemia

•Acute leukemia

•Infectious mononucleosis

•Wegener granulomatosis

•Acute necrotizing ulcerative gingivitis and stomatitis

Treatment

Basic Guidelines

•Potential causative drug must be discontinued.

•High level of oral hygiene must be maintained.

•Any dental and gingival treatment during the acute phase of the disease should be avoided.

•The systemic treatment must be provided by a specialist.

Suggested Therapies

Topical Treatment

The use of mouthwashes such as 0.1 % chlorhex-idine gluconate and oxygen peroxide may reduce the severity of the oral lesions.

Systemic Treatment

•Systemic administration of appropriate broad-spectrum antibiotics in doses and duration proportional to the severity of the bacterial infection.

•Granulocyte-macrophage colony-stimulating factor (GM-CSF) or recombinant human granulocyte colony-stimulating factor (rhG-CSF) is effective in shortening the recovery time in patients with agranulocytosis.

References

Friedman I, Klepfish A, Miller EB, et al. Agranulocytosis in Sjögren’s syndrome: Two case reports and analysis of 11 additional reported cases. Semin Arthritis Rheum 2002;31:338–345.

Welte K, Dale D, Pathophysiology and treatment of severe chronic neutropenia. Ann Hematol 1996;72:158.