Caplacizumab Use in a TTP Case Unresponsive to Conventional Therapy.
BLOOD AND IMMUNE DISORDER
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JournalIrish medical journal
AbstractIntroduction Thrombotic Thrombocytopenic Purpura (TTP) is a rare but life-threatening disorder caused by severely reduced activity of ADAMTS13, causing platelet adhesion and formation of small-vessel platelet-rich thrombi, thrombocytopenia, and microangiopathic haemolytic anaemia. Diagnosis A 48-year-old female presented with acute generalized petechial rash, bruises, and fatigue. Bloods revealed thrombocytopenia, anaemia, 10% schistocytes. Her plasmic score was seven, and ADAMT13 was <5. Treatment Patient initially responded to plasma exchange and steroids, but thrombocytopenia recurred on day six of treatment, needing the addition of further immunosuppressive drugs and Caplacizumab. Conclusion TTP cases unresponsive to conventional regimens can represent a challenging situation; however, poor outcomes could potentially be avoided with a novel therapy like Caplacizumab. In our patient, this medication was well tolerated, and platelet count normalized after two days of its introduction.
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