Biomaterial Database

Caplacizumab for congenital thrombotic thrombocytopenic purpura. 2022

Aaron Boothby, and Marshall Mazepa

Department of Medicine, University of Minnesota Medical School, Minneapolis, Minnesota, USA.

Caplacizumab rapidly increased platelet count which likely shorted the hospital stay and reduced plasma transfusion requirement.

UI	MeSH Term	Description	Entries
D010949	Plasma	The residual portion of BLOOD that is left after removal of BLOOD CELLS by CENTRIFUGATION without prior BLOOD COAGULATION.	Blood Plasma,Fresh Frozen Plasma,Blood Plasmas,Fresh Frozen Plasmas,Frozen Plasma, Fresh,Frozen Plasmas, Fresh,Plasma, Blood,Plasma, Fresh Frozen,Plasmas,Plasmas, Blood,Plasmas, Fresh Frozen
D010951	Plasma Exchange	Removal of plasma and replacement with various fluids, e.g., fresh frozen plasma, plasma protein fractions (PPF), albumin preparations, dextran solutions, saline. Used in treatment of autoimmune diseases, immune complex diseases, diseases of excess plasma factors, and other conditions.	Exchange, Plasma,Exchanges, Plasma,Plasma Exchanges
D011697	Purpura, Thrombotic Thrombocytopenic	An acquired, congenital, or familial disorder caused by PLATELET AGGREGATION with THROMBOSIS in terminal arterioles and capillaries. Clinical features include THROMBOCYTOPENIA; HEMOLYTIC ANEMIA; AZOTEMIA; FEVER; and thrombotic microangiopathy. The classical form also includes neurological symptoms and end-organ damage, such as RENAL FAILURE. Mutations in the ADAMTS13 PROTEIN gene have been identified in familial cases.	Moschkowitz Disease,Purpura, Thrombotic Thrombopenic,Thrombotic Thrombocytopenic Purpura, Congenital,Thrombotic Thrombocytopenic Purpura, Familial,Congenital Thrombotic Thrombocytopenic Purpura,Familial Thrombotic Thrombocytopenia Purpura,Familial Thrombotic Thrombocytopenic Purpura,Microangiopathic Hemolytic Anemia, Congenital,Moschcowitz Disease,Schulman-Upshaw Syndrome,Thrombotic Microangiopathy, Familial,Thrombotic Thrombocytopenic Purpura,Upshaw Factor, Deficiency of,Upshaw-Schulman Syndrome,Familial Thrombotic Microangiopathy,Microangiopathy, Familial Thrombotic,Schulman Upshaw Syndrome,Thrombocytopenic Purpura, Thrombotic,Thrombopenic Purpura, Thrombotic,Thrombotic Thrombopenic Purpura,Upshaw Schulman Syndrome
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000071120	ADAMTS13 Protein	An ADAMTS protease that contains eight thrombospondin (TS) motifs. It cleaves VON WILLEBRAND FACTOR to control vWF-mediated THROMBOSIS. Mutations in the ADAMTS13 gene have been identified in familial cases of PURPURA, THROMBOTIC THROMBOCYTOPENIC and defects in ADAMTS13 activity are associated with MYOCARDIAL INFARCTION; BRAIN ISCHEMIA; PRE-ECLAMPSIA; and MALARIA.	A Disintegrin and Metalloproteinase with Thrombospondin Motifs 13 Protein,ADAMTS-13 Protein,ADAMTS13 Protease,vWF-Cleaving Protease,von Willebrand Factor-Cleaving Protease,ADAMTS 13 Protein,vWF Cleaving Protease,von Willebrand Factor Cleaving Protease
D014841	von Willebrand Factor	A high-molecular-weight plasma protein, produced by endothelial cells and megakaryocytes, that is part of the factor VIII/von Willebrand factor complex. The von Willebrand factor has receptors for collagen, platelets, and ristocetin activity as well as the immunologically distinct antigenic determinants. It functions in adhesion of platelets to collagen and hemostatic plug formation. The prolonged bleeding time in VON WILLEBRAND DISEASES is due to the deficiency of this factor.	Factor VIII-Related Antigen,Factor VIIIR-Ag,Factor VIIIR-RCo,Plasma Factor VIII Complex,Ristocetin Cofactor,Ristocetin-Willebrand Factor,von Willebrand Protein,Factor VIII Related Antigen,Factor VIIIR Ag,Factor VIIIR RCo,Ristocetin Willebrand Factor
D016913	Blood Component Transfusion	The transfer of blood components such as erythrocytes, leukocytes, platelets, and plasma from a donor to a recipient or back to the donor. This process differs from the procedures undertaken in PLASMAPHERESIS and types of CYTAPHERESIS; (PLATELETPHERESIS and LEUKAPHERESIS) where, following the removal of plasma or the specific cell components, the remainder is transfused back to the donor.	Blood Component Transfusions,Component Transfusion, Blood,Component Transfusions, Blood,Transfusion, Blood Component,Transfusions, Blood Component
D061905	Single-Domain Antibodies	An immunoglobulin fragment composed of one variable domain from an IMMUNOGLOBULIN HEAVY CHAIN or IMMUNOGLOBULIN LIGHT CHAIN.	Single-Domain Antibody,VHH Antibodies,VHH Antibody,Immunoglobulin VH Domain Fragments,Immunoglobulin VL Domain Fragments,Nanobodies,VH Domain Fragments,VHH Fragments,VHH Immunoglobulin Fragments,VL Domain Fragments,VNAR Fragments,VNAR Immunoglobulin Fragments,Antibody, Single-Domain,Antibody, VHH,Single Domain Antibody