CE ARTICLE

patient first begins to ambulate. In moderately affected individuals, the diagnosis may be delayed until 2 to 5 years of age. The knees are the most prominent sites of spontaneous bleeds, followed by the elbows, ankles, shoulders and hips.

CASE REPORT

A 24-year-old male presents to the emergency department with chief complaint of continued bleeding from the oral cavity for approximately 24 hours. He states that he was driving home and noted bleeding from his up- per right jaw. The patient denied any history of pain, swelling or trauma. He denies any past or current medical treatments. He denied usage of over- the-counter medication, supplements or prescribed medication. He reports removal of a pilonidal cyst a few years back. He denies any allergies. He has had a one-pack per day smoking his- tory for the past eight years. At this time, imaging is ordered by the attend- ing emergency room physician (Figure 1). The radiologist read of the ortho- pantomogram finds no acute process. The physician provides a diagnosis of a dental abscess and discharges the patient home with antibiotics and pain medication.

Figure 1) Orthopantogram ordered with radiologist reading of non-opacified maxillary sinuses without evidence of air-fluid levels. No bony fractures notes. There is a small focus of bony density adjacent to the tip of the roots of the right mandibular second molar consistent with enostosis. Asymptomatic impacted teeth #17 and #32. Figure 2) Ooze is noted from the lingual gingiva of tooth #2 and #3. Figure 3) Lab values show grossly abnormal PTT value with elevated INR and PT. Blood chemistry and complete blood count studies are within normal values. Figure 4) Coagulation factor assays reveal deficiency in Factor VIII.

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The patient is ultimately admitted to hematology service for work up and treatment of prolonged bleeding. The patient is placed on Tranexamic acid mouth rinses to aid in clot reten- tion. The bleeding is controlled and he is discharged to be treated as an outpatient.

Further work up for coagulation fac- tors reveals a deficiency of factor VIII, revealing diagnosis of hemophilia A (Figure 4).

DISCUSSION 

Hemophilia A is the most common he- reditary coagulation disorder. It is an X chromosome, sex-linked disease and, hence, mostly affects males. However, it may be seen in females, by mating of a male hemophiliac with a female car- rier, or in carrier females afflicted with Turner’s syndrome (XO karyotype)5

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The severity of hemophilia can be clas- sified as severe (less than 1 percent of normal factor VIII activity), moderate (1 to 5 percent of normal activity), or mild as in our case presentation (5 to 25 percent of normal activity). Patients must retain 30 percent of coagulation factor for normal clotting.

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The patient returned the next day with continued bleed and tachycardia. The at- tending physician at this time requests an oral and maxillofacial consult. Basic labs were requested at this time. Further ques- tioning of the patient reveals a family history of hemophilia, but is unsure of type. The patient has not seen a dentist in quite some time but does not recall prolonged bleeding after treatments. When questioned about his pilonidal cystectomy procedure, he does not recall complications or excessive bleeding. In addition to his smoking habit, he admits to social alcohol use and denies any street drug use. He works as a construction worker and is having domestic problems due to lack of work.

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An exam reveals a well-developed male in no apparent distress. No ecchymosis of the extremities or trunk of body. Bleeding is in noted from lingual gingiva in area of #2 and #3, with a slow ooze in character (Figure 2). The teeth are non-mobile and non-painful to percussion. No appreciated decayed sur- faces. No parulis or fistulas are noted. A well adapted occlusal filling on tooth #3 is noted.

The requested laboratory studies reveal normal blood chemistry and complete blood count (CBC) studies. It is noted that the Partial Thromboplastin Time (PTT) is grossly elevated. In light of a clinical presenta- tion of spontaneous bleed, a family history of hemophilia and elevated PTT, it was recommended for a hematology consult.

Screening for these patients normally starts with a coagulation study that includes PTT, PT and an International

Normalized Ratio (INR). Patients afflicted with hemophilia would normally see an elevated PTT with a normal PT time. PTT is a measure of the intrinsic coagulation cascade, or now more commonly known as the contact activation pathway. It evaluates deficiencies in factors such as II, V, VIII, IX, X, XI and XII. Normally PTT is within 25 to 35 seconds, but standards may vary by test- ing facility. An elevated PTT would trigger suspicion for bleeding conditions to include von Willebrand Disease, Hemophilia A and B. However, it must be noted that more mild hemophiliacs (e.g., those with Factor VIII greater than 30 percent) may not induce a prolonged PTT time. If hemophilia is clini-