Scott Syndrome

Scott Syndrome
Jan 26, 2021 1:14pm

I [Geo] provide a quarterly series of hemostasis lectures for UAB's pathology residents and fellows. Last week, our transfusion medicine fellow, Richard Godby, MD, asked if I'd heard of "Scott syndrome." This was new to me and also to two of my colleagues. Dr. Godby provided me with this Wikipedia link [click] that describes the syndrome. Scott syndrome is a rare autosomal recessive platelet membrane disorder that prevents phosphatidyl serine from migrating to the outer membrane layer upon activation. With reduced phosphatidyl serine the platelet-dependent coagulation complexes assemble poorly, leading to reduced thrombin generation. For detail, see Jeske W, DeChristopher P, Qualitative disorders of platelets and vasculature in Keohane EM, Otto CN, Walenga JM. Rodak's Hematology: Clinical Principles and Applications. Elsevier, 2020 , and also Zwaal RFA, Comfurius P, Bevers EM. Scott syndrome, a bleeding disorder caused by defective scrambling of membrane phospholipids. Biochimica et Biophysica Acta 2004; 1636: 119–28. Many thanks to Dr. Godby for the heads-up.

0 Comments

I [Geo] provide a quarterly series of hemostasis lectures for UAB's pathology residents and fellows. Last week, our transfusion medicine fellow, Richard Godby, MD, asked if I'd heard of "Scott syndrome." This was new to me and also to two of my colleagues. Dr. Godby provided me with this Wikipedia link [click] that describes the syndrome. Scott syndrome is a rare autosomal recessive platelet membrane disorder that prevents phosphatidyl serine from migrating to the outer membrane layer upon activation. With reduced phosphatidyl serine the platelet-dependent coagulation complexes assemble poorly, leading to reduced thrombin generation. For detail, see Jeske W, DeChristopher P, Qualitative disorders of platelets and vasculature in Keohane EM, Otto CN, Walenga JM. Rodak's Hematology: Clinical Principles and Applications. Elsevier, 2020 , and also Zwaal RFA, Comfurius P, Bevers EM. Scott syndrome, a bleeding disorder caused by defective scrambling of membrane phospholipids. Biochimica et Biophysica Acta 2004; 1636: 119–28. Many thanks to Dr. Godby for the heads-up.

Leave A Comment

You must be logged in to Comment - Sign In