Bleeding disorders

In this case discussion, Rajiv Pruthi, MD and Taha Bat, MD walk through a patient with acquired hemophilia A, a rare bleeding disorder caused by autoantibodies against clotting factor VIII. They review how the diagnosis is reached (especially distinguishing it from congenital hemophilia), discuss the challenges in management (bleeding control, immunosuppression, and inhibitor eradication), and present clinical decision points based on published evidence and expert experience.

Key Learning Points:

• Patients often present with new, unexplained bleeding in adulthood — especially soft tissue, mucosal, or spontaneous bleeding — with no prior personal or family history of bleeding.
• Key labs include prolonged aPTT that does not correct on mixing studies, measurement of factor VIII levels (low), and detection/quantification of FVIII inhibitors (Bethesda assay or variations).
• In active bleeding, bypassing agents (e.g. recombinant activated factor VII or activated prothrombin complex concentrates) are used to control hemorrhage, rather than conventional factor VIII replacement that is neutralized by inhibitors.
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In this session, Adam Cuker, MD and Taha Bat, MD present a case-oriented dive into immune thrombocytopenia (ITP). They review the pathophysiology of platelet autoantibody-mediated destruction, the diagnostic workup focused largely on exclusion, and a tiered approach to therapy based on severity, bleeding risk, and patient factors.

Key Learning Points:

• ITP is characterized by autoantibody-mediated platelet destruction and sometimes impaired platelet production by megakaryocytes.
• Diagnosis is one of exclusion: labs, peripheral smear, ruling out secondary causes (e.g. lupus, HIV, hepatitis).
• Not all patients need immediate treatment; decisions consider platelet count, bleeding symptoms, and patient comorbidities.
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In this session, Shannon Meeks, MD, and Ibrahim Ibrahim, MD review current best practices for managing Hemophilia A. They emphasize the move toward individualized, preventive care, noting that well-designed prophylactic therapycan significantly reduce bleeding events and prevent long-term joint damage. The speakers highlight the ongoing need to monitor for complications, particularly inhibitor development and joint deterioration, even with modern treatments. They also stress that optimal outcomes depend on multidisciplinary care, integrating hematology, nursing, physical therapy, and patient-education support to address both clinical and psychosocial needs.

Key Learning Points:

• Updated diagnostic and treatment strategies for Hemophilia A.
• Importance of individualized care plans — therapy should be tailored depending on patient’s severity, bleeding risk, and lifestyle.
• Role of prophylactic therapy and prevention: continuing preventive treatment can significantly reduce bleeding episodes and long-term complications.
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