PEDIATRICDENTISTRY/Copyright ’9 1981by TheAmerican Academy of Pedodontics/Vol. 3, No. 3 CAS Dental managementof idiopathic aplastic anemia: report of a case JamesE. Jones, DMD, MS ThomasD. Coates, MD Charles Poland, DDS Abstract since the disease has been knownto occur at any age. Usually, the onset is gradual, but acute fulminating Aplastic anemiais a serious and often fatal hematological ,4 disorder characterized by hypoplastic bone marrowand cases have been reported? The mortality in severe peripheralpancytopenia. Epistaxis, oral lesions and gingival cases is more than 50 percent during the first year and hemorrhageoften necessitates multiple platelet transfusions may be greater than 70 percent at five years? Aplastic in these patients. The use of aminocaproicacid to control anemia is normochromic and normocytic, and mani- hemorrhagicepisodes has been especially beneficial in fests itself as a pancytopenia. The bone marrow patients with bone marrowhypoplasia as they often become is devoid of megakaryocytes, myeloid and erythroid refractory to repeatedtransfusions. In this case precursors? presentation, a 15-year-old black female with idiopathic Clinical signs and symptoms include: 1) severe aplastic anemiawas treated with a combination of modalities including initial platelet transfusion, oral hygiene weakness and dyspnea even after mild physical exer- tion, 2) pallor of the skin, 3) numbnessand tingling instruction, dental prophylaxis and systemic aminocaproic acid. The health of the oral tissues greatly improved the extremities, 4) decreased resistance to infection, following this regimen. and 5) petechiae of the skin and mucous membranes; These clinical manifestations are caused by the inabil- Introduction ity of the hematopoietic system to deliver enough red Aplastic anemia is a serious and often fatal hema- cells, white cells and platelets to the peripheral circu- tologic disorder characterized by hypoplastic bone lation. The specific clinical picture varies according to marrow and peripheral pancytopenia. Recently, the the cell line predominantly affected. Oral signs in- use of systemic aminocaproic acid a in these patients clude: 1) spontaneous bleeding from the mucous mem- has demonstrated positive results in the control of branes, 2) petechiae, 3) purpuric spots, and 4) frank oral hemorrhagic episodes, thus reducing the need for hematomas of the mucosa, pharnyx and gingiva. multiple platelet transfusions.’ In this case presenta- Aplastic anemia is generally recognized in two tion, a 15-year-old black female with severe idiopathic forms: idiopathic and secondary. Idiopathic aplastic aplastic anemia was treated with a combination of anemia affects young adults, progresses rapidly and is modalities, including initial platelet transfusion, oral usually fatal. This form accounts for approximately 60 hygiene instruction, dental prophylaxis and systemic percent of the reported cases? Secondary aplastic aminocaproic acid. The oral tissues appeared clinically anemia is of knownetiology and can affect individuals healthy following this regimen. at any age. The prognosis, once again, is poor even if the causative agent is identified. Amongthe agents as- Literature Review sociated with secondary aplastic anemia are: ionizing Aplastic anemia was first described in 1888 by Ehr- radiation, ~ Atabrine/° chloramphenicol/u2 benezene/TM lich as a rapidly fatal hematologic disease seen in gold compounds,’~ viral hepatitis, and miliary tubercu- young adults between the ages of 15 and 30. 2 Today, losis? ,~ Secondaryaplastic anemia accounts for approxi- the concept of aplastic anemia has been broadened mately 40 percent of reported cases? Accepted:December 29, 1980 Report of Case aNon-proprietaryname and trademarksof drug: amniocaproicacid A 15-year-old black female was referred to the -- Amicar,Cuprocid. James Whitcomb Riley Hospital for Children in PEDIATRICDENTISTRY: Volume 3, Number3 267 noted. Subsequent normal 612 and folate levels were documented. A sucrose hemolysis test proved nega- tive. In the absence of historical evidence for marrow toxins and the fact that the physical findings were not consistent with familial aplastic anemia, the diagnosis of idiopathic aplastic anemia was made. A histo- compatible donor was unavailable for marrow trans- plantation. The patient was discharged from the hospi- tal to her parents five days after admission. Subsequent attempts at treatment with high dose corticosteroids Figure 1. Initial intraoral view demonstrating gingivitis and spontaneous gingival hemorrhage. and antithymocyte globulin were unsuccessful. At the request of the hematology service, the pa- March, 1979, with complaints of malaise and pro- tient was seen in the dental clinic of Riley Hospital on longed epistaxis. The patient denied any episodes of October 18, 1979 for evaluation of gingival hemor- jaundice or hematuria. The family history was nega- rhage and oral lesions. Her oral temperature was 38.9° tive for sickle cell anemia or Fanconi's anemia. The C, hematocrit 20%, and a platelet count of 1,000 patient had been transfused with 5 units of random cu mm. donor platelets and packed cells prior to arrival at Oral and Radiographic Examination Riley Hospital. Oral examination demonstrated generalized gingivi- Physical examination revealed a well-developed tis with spontaneous gingival hemorrhage (Figure 1). black female in no acute distress. Heart rate was 80, Periodontal examination demonstrated no pocket for- respiratory rate 18, weight 38.1 Kg. The examination mation greater than three millimeters. Multiple was unremarkable with the exception of mucosal and round, raised lesions approximately 3 mm in diameter petechial hemorrhage. Specifically, there was no hepa- were present on the anterior half of the tongue (Figure tosplenomegaly or lymphadenopathy. No abnormality 2). Bilateral lesions approximately 1 cm in diameter of the digits was noted. The patient was admitted to were distal to the second permanent molars on the the hospital for evaluation. Admission laboratory data buccal mucosa (Figure 3). There was no history of oral were as follows: Hemoglobin 7.6 gr.%, hematocrit 24%, trauma. Radiographic examination, including pano- white cell count 2,400/cu mm, platelets 10,000/cu mm, rex, bite-wing and two maxillary anterior periapical PT 11.5 seconds, PTT 23.0 seconds, SCOT 20, SPOT radiographs were negative for pathology. 19, and Alkaline Phosphatase 91. Antinuclear anti- The patient reported severe pain during tooth- body was negative. brushing and had not been practicing regular oral The patient was transfused with packed red cells, hygiene in the past. At this time the patient was read- whole blood and five units of random donor platelets mitted to the hospital for dental evaluation and treat- shortly after admission. A repeat hemoglobin was 9.1 ment of the oral condition. gr.%. A bone aspiration and biopsy were performed. Initial Dental Treatment These demonstrated a marked decrease in precursors The patient received two transfusions of 10 units of all cell lines. Prominent mast cells and plasma cells each of platelets. The platelets were matched by were noted. There were no megaloblastic changes major blood groups only. This brought her platelet Figure 2. Multiple round, raised lesions on the anterior half of the tongue (left). Figure 3. Intraoral view of lesion on patient's left buccal mucosa (right). 268 IDIOPATHIC APLASTIC ANEMIA: Jones, Coates, and Poland count to 190,000/cu mm. At this time, definitive den- persistent, severe thrombocytopenia (2,000/cu mm), tal treatment was initiated. Oral hygiene instructions and periodic epistaxis. The need for platelets had been were given to the patient and her parents. The need reduced to approximately 5 units per month. Gingival for daily oral care was stressed and the patient inflammation and oral lesions decreased within two demonstrated that she could remove plaque from all weeks. The patient did experience a mild candidosis surfaces of her teeth using a toothbrush and dental during a three-day period (Figure 4-6). The patient floss. A thorough debridement of the gingiva and a maintained meticulous oral hygiene and was followed prophylaxis of the teeth were completed. Nitrous regularly in the dental clinic for observation and recall oxide-oxygen analgesia was used to reduce the pa- examinations. During the fourth month of amino- tient's anxiety concerning dental treatment. The caproic acid therapy, the patient experienced signifi- patient was seen daily in the dental clinic for the cant episodes of nasopharyngeal hemorrhage requiring remainder of her admission for evaluation and rein- hospitalization. This was controlled by adjusting the forcement of her oral hygiene. Upon her release from dosage of aminocaproic acid to 20 gm/day (5.0 gm the hospital, five days after initial dental treatment, q.i.d.), nasal packing and platelet transfusions. She is the gingivitis had been reduced, although some spon- being maintained at this dosage. taneous gingival hemorrhage continued and the oral lesions remained essentially unchanged. This was thought to be secondary to her thrombocytopenia. The patient was evaluated in the dental clinic on November 26, 1979. She was again admitted to the hospital for severe, prolonged epistaxis and gingival hemorrhage. Her hematocrit was 30% and her platelet count was 2,000/cu mm. She received 10 units of platelets. The patient complained of moderate intra- oral pain and was given two Tylenol #3b tablets one hour before dental treatment,