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Cracking the Code: How Doctors Diagnose Acquired Hemophilia A
Published: Aug 06, 2024
Diagnosing acquired hemophilia A can be like solving a medical mystery. This rare bleeding disorder often stumps doctors unfamiliar with its sneaky symptoms. Let's unravel the diagnostic process step by step.
The First Clues
The journey usually begins when a patient presents with unexplained, severe bruising or bleeding. Unlike inherited hemophilia, there's typically no family history. The bleeding pattern is different too - large, soft tissue bruises are common, while joint bleeds are rare. These unusual symptoms often prompt doctors to investigate further.
Blood Tests Tell the Tale
The key to diagnosis lies in specialized blood tests. The first red flag is often a prolonged activated partial thromboplastin time (aPTT), while the prothrombin time (PT) remains normal. This pattern suggests a problem in the intrinsic clotting pathway. The next crucial step is a mixing study, where patient plasma is mixed with normal plasma. If the aPTT doesn't correct, it indicates the presence of an inhibitor.
Pinpointing the Problem
Once an inhibitor is suspected, doctors perform specific factor assays. In acquired hemophilia A, factor VIII activity is typically low. The final piece of the puzzle is the Bethesda assay, which measures the strength of the factor VIII inhibitor. This test not only confirms the diagnosis but also helps guide treatment decisions.
Ruling Out Other Causes
Diagnosis also involves excluding other conditions that can cause similar symptoms. This might include tests for liver function, vitamin K deficiency, or other clotting factor deficiencies. In some cases, doctors may look for underlying conditions associated with acquired hemophilia A, such as autoimmune diseases or cancers.
Frequently Asked Questions
It can vary, but specialized tests may take several days.
No, diagnosis is typically made through blood tests.
Standard tests may show abnormalities, but specific tests are needed for diagnosis.
No, as it's not an inherited condition.
Very accurate when proper tests are performed.
The Bottom Line
While diagnosing acquired hemophilia A can be challenging, a systematic approach using specialized blood tests can unlock the mystery and guide life-saving treatment.
References
- Collins PW, et al. Diagnosis and management of acquired coagulation inhibitors: a guideline from UKHCDO. Br J Haematol. 2013;162(6):758-773.
- Kruse-Jarres R, et al. Acquired hemophilia A: Updated review of evidence and treatment guidance. Am J Hematol. 2017;92(7):695-705.
This article has been reviewed for accuracy by one of the licensed medical doctors working for Doctronic. Always discuss health information with your healthcare provider.
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