This guideline complements previous guidelines, focussing on the laboratory methods associated with testing for deficiency of the metalloproteinase ADAMTS13.

This document is intended as a guide for laboratories in the United Kingdom that may receive requests for assays for the metalloproteinase ADAMTS13 and covers aspects of testing that should be considered by all haematology laboratories. In the United Kingdom, assays for ADAMTS13 activity, anti-ADAMTS13 antibodies and ADAMTS13 antigen for the diagnosis or exclusion of thrombotic thrombocytopenic purpura (TTP) are usually performed in regional TTP centres. However, it is recognised that most haematology laboratories receive occasional requests for assays for ADAMTS13, even if they are not regional TTP centres.

Declaration of Interests

The BSH paid the expenses incurred during the writing of this guidance. All authors have made a declaration of interests to the BSH, which may be viewed on request. The following authors have declared reimbursement for travel, speaking or consultancy fees: SM—Werfen and Randox; TP—Sysmex; RG—Roche and Sobi; SP—Sysmex, Pfizer and Roche-Chugai. The following members of the writing group list initials have no conflicts of interest to declare: DS; RF; PVJ; CL.