Glossary

ULvWF

One of my favorite abbreviations in the whole world! ULvWF stands for “ultra-large von Willebrand factor” (but in my mind, I will always think of it as “unusually large vWF”). In most cases of thrombotic thrombocytopenic purpura (TTP), the underlying problem is a deficiency of the ADAMTS13 enzyme. ADAMTS13 normally acts to “trim” vWF molecules into smaller fragments. No ADAMTS13 means no trimming, so the molecules stay “large” (OK, “ULTRA-LARGE!”). The ULvWF multimers attract platelets, which form thrombi in small blood vessels and lead to vascular and red blood cell damage (both important parts of the pathophysiology of TTP).

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