We searched the MEDLINE (via PubMed), LILACS (via BVS) and UpToDate databases for the terms: “Congenital Thrombotic Thrombocytopenic Purpura”, “Hereditary Thrombotic Thrombocytopenic Purpura”, “Familial Thrombotic Thrombocytopenic Purpura”, “Upshaw-Schulman syndrome”, “Thrombotic Thrombocytopenic Purpura”, “Microangiopathic Hemolytic Anemia”, “Pregnancy” and “ADAMTS13” (Table 1). Here, ADAMTS13 is linked to congenital thrombotic thrombocytopenic purpura.