Recombinant ADAMTS13 for Immune Thrombotic Thrombocytopenic Purpura.

Autor:	Bendapudi PK; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Foy BH; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Mueller SB; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Liu J; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Feingold LM; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Burke KE; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Cruz W; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Chen MY; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Lau ES; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Goldberg RL; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Tatake I; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Wilkinson SC; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Carney BJ; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Stone JR; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Park D; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Avelino AR; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Hassan S; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Andrzejewski C; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Ruby KN; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Friedman KD; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Brunker PAR; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Leaf RK; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Higgins J; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Dzik WH; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Stefely JA; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.)., Makar RS; From the Blood Transfusion Service (P.K.B., K.N.R., P.A.R.B., W.H.D., J.A.S., R.S.M.), the Division of Hematology (P.K.B., R.K.L., W.H.D.), the Department of Pathology (B.H.F., J.L., M.Y.C., J.R.S., J.H.), and the Division of Cardiology (E.S.L., R.L.G.), Massachusetts General Hospital, the Division of Hemostasis and Thrombosis (P.K.B., L.M.F., K.E.B., I.T., S.C.W.) and the Division of Hematology and Apheresis Service (B.J.C.), Beth Israel Deaconess Medical Center, and Harvard Medical School (P.K.B., J.L., M.Y.C., E.S.L., R.L.G., I.T., S.C.W., B.J.C., J.R.S., K.N.R., P.A.R.B., R.K.L., J.H., W.H.D., J.A.S., R.S.M.), Boston, the Broad Institute of MIT and Harvard, Cambridge (P.K.B.), the Division of Hematology and Oncology, Lahey Hospital and Medical Center, Burlington (D.P.), and the Department of Hematology and Clinical Oncology (A.R.A.) and the Department of Pathology, Transfusion/Apheresis Medicine Services (S.H., C.A.), UMass Chan Medical School-Baystate Health, Springfield - all in Massachusetts; the Department of Laboratory Medicine and Pathology, University of Washington, Seattle (B.H.F.); and Versiti Blood Center of Wisconsin, Milwaukee (S.B.M., W.C., K.D.F.).
Jazyk:	angličtina
Zdroj:	The New England journal of medicine [N Engl J Med] 2024 May 09; Vol. 390 (18), pp. 1690-1698.
DOI:	10.1056/NEJMoa2402567
Abstrakt:	In patients with immune thrombotic thrombocytopenic purpura (iTTP), autoantibodies against the metalloprotease ADAMTS13 lead to catastrophic microvascular thrombosis. However, the potential benefits of recombinant human ADAMTS13 (rADAMTS13) in patients with iTTP remain unknown. Here, we report the clinical use of rADAMTS13, which resulted in the rapid suppression of disease activity and complete recovery in a critically ill patient whose condition had proved to be refractory to all available treatments. We also show that rADAMTS13 causes immune complex formation, which saturates the autoantibody and may promote its clearance. Our data support the role of rADAMTS13 as a novel adjunctive therapy in patients with iTTP. (Copyright © 2024 Massachusetts Medical Society.)
Databáze:	MEDLINE
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