An 18-year-old man develops excessive bleeding 2 hours after wisdom tooth extraction. He has a history of easy bruising after playing sports, and of minor cuts that rebleed. His examination is normal, except for the tooth extraction site, which is still oozing blood. His hemoglobin is 14.8 g/dL, platelets 230,000 mL, PT is normal, and partial thromboplastin time (PTT) is elevated. A bleeding time is normal, factor VIII level is reduced, factor IX is normal, and ristocetin cofactor assay is normal.For the above patient with a bleeding disorder, select the most likely diagnosis.

## **Core Concept**
The patient's presentation of excessive bleeding after a surgical procedure, a history of easy bruising, and prolonged partial thromboplastin time (PTT) with a normal prothrombin time (PT) and bleeding time, points towards a disorder of the intrinsic coagulation pathway. This pathway involves factors VIII, IX, XI, and XII. The key laboratory findings here are a reduced factor VIII level and a normal factor IX level.

## **Why the Correct Answer is Right**
The patient's clinical presentation and laboratory findings are consistent with **Hemophilia A**, also known as classic hemophilia. This condition is caused by a deficiency in factor VIII, a crucial component of the intrinsic coagulation pathway. The normal factor IX level rules out **Hemophilia B** (Christmas disease), which is caused by a deficiency in factor IX. The normal bleeding time and ristocetin cofactor assay also help to rule out disorders of platelet function or von Willebrand disease, as the ristocetin cofactor assay would be expected to be low in the latter condition.

## **Why Each Wrong Option is Incorrect**
- **Option A:** This option would correspond to a diagnosis that does not match the provided clinical and laboratory findings. Without the specific text of option A, we can infer that it likely represents a condition not supported by the evidence, such as a factor IX deficiency (Hemophilia B), which is not consistent with the normal factor IX level.
- **Option B:** Similarly, this option does not align with the patient's presentation. It could potentially represent another coagulation disorder not supported by the lab findings, such as von Willebrand disease, which would have an abnormal ristocetin cofactor assay.
- **Option D:** This option would represent a diagnosis that is not consistent with the provided information. Given the specifics of the case, it might suggest a different coagulation factor deficiency or a disorder of platelet function, neither of which fits the patient's profile.

## **Clinical Pearl / High-Yield Fact**
A key clinical pearl in this case is that patients with Hemophilia A and B typically have normal bleeding times because their platelet function is intact; the defect lies in the coagulation cascade. This is why the bleeding time is normal in this patient. Additionally, the fact that Hemophilia A is more common than Hemophilia B and that it is caused by a deficiency in factor VIII is crucial for diagnosis.

## **Correct Answer:** C. Hemophilia A.

✓ Correct Answer: B. hemophilia A