Conservative management for a patient with aplastic anemia without use of blood products. Case report.

Aplastic anemia, the failure of the pluripotent stem cell system, is characterized by pancytopenia and hypoplasia of bone marrow. The patient exhibits signs and symptoms of neutropenia and thrombocytopenia. Though the disease is rare in children, when it is seen, the peak age of incidence is between 3 and 5 years. The acquired form may occur as a toxic reaction to medications (e.g., antiinflammatory, antiepileptic, antimalarial, or antidiabetic drugs), chemotherapeutic agents, or chemicals (e.g., solvents and insecticides). It also may occur as a complication of infection, most notably hepatitis, or in association with early manifestations of leukemia. However, in half of the patients, no causative agent can be identified (Silver et al. 1987). The most current classification of aplastic anemia consists of those patients with a severe form of the disease and those with a mild to moderate form. Severe aplasia is defined by granulocyte counts of less than 0.5 x 109/L, platelets less than 20 x 109/L and reticulocytes less than 20 x 109/L (Champlin 1981). A ~latelet count of 20 x 109/L is equivalent to 20,000/mm . The moderate form of the disease is defined by at least two of the following criteria: granulocytes less than 1.0 x 109/L; platelets less than 50 x 109 / L (50,000 / mm3); and reticulocytes less than 60 x 109/L (Champlin et al. 1983). Clinical signs and symptoms include pallor, fatigue, fever and an increased tendency to bleed. Treatment varies based on whether the disease is the acquired or the congenital form, the age of the patient, and the severity of the disease. The congenital form is less common and generally is treated with blood transfusions and antibiotics, along with androgenic steroids or corticosteroid therapy, to reduce the patient’s tendency to bruise and bleed. Most of these patients require continuous therapy to control the disease process (Behrman and Vaughan 1987). Treatment of the congenital or acquired forms also may consist of a bone marrow transplant from a sibling with human leukocyte antigen-compatible marrow. Some feel that this procedure yields better results in younger patients and in more severe cases (Champlin et al. 1983). In acquired cases, can increase the survival rate by 50-70%. Fanconi’s anemia, or the congenital familial form of aplastic anemia, is believed to be inherited as an autosomal recessive trait. Though about two-thirds of these children present with other congenital anomalies (e.g., microcephaly, short stature, and hyperpigmentation of the skin) the pancytopenia usually is not present at birth or during infancy. Bruising secondary to the thrombocytopenia is usually the first clinical presentation; it tends to appear between 1-1/2 to 22 years of age, with an average age of 6-8 years (Behrman and Vaughan 1987). Oral manifestations secondary to neutropenia and thrombocytopenia include purpura, spontaneous gingival bleeding, severe ulcerative stomatitis, and/or pharyngitis. Lesions may become large and necrotic, and have an accompanying foul odor. Cervical and submandibular lymphadenopathy also are common (Rose and Kaye 1983). Dental management often requires costly and timeconsuming administration of blood products before treatment. Often, parents do not want blood products administered unless absolutely necessary. The following case discusses the dental management of a pediatric patient with aplastic anemia for whom dental treatment was provided under general anesthesia on an outpatient basis without the administration of platelets.