Moskowitz syndrome manifesting as recurrent large vessel strokes in a young male-An original research paper
Keywords:
TTP - Thrombotic Thrombocytopenic Purpura, vWF - von Willebrand Factor, PEX- Plasma Exchange Therapy, ADAMTS13 - A Disintegrin and Metalloprotease with Thrombospondin type 1 motif member 13 HUS - Hemolytic Uremic Syndrome, PLASMIC- Platelet count, hemolysis, absent kidney injury absence of active cancer presence of mycoplasma, and no other cause of thrombocytopenia score, ISTH - International Society on Thrombosis and HaemostasisAbstract
Background: Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening condition characterized by microthrombus formation due to a deficiency of ADAMTS13, which cleaves von Willebrand factor multimers. This deficiency leads to widespread microangiopathic hemolytic anemia and multiple organ involvement, particularly affecting the brain, heart, and kidneys. TTP can present atypically and poses a diagnostic challenge, especially in young patients with neurological symptoms and thrombocytopenia. Early diagnosis and prompt initiation of plasma exchange therapy (PEX) and steroids are critical to reduce mortality.
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