ABSTRACT
Immune thrombotic thrombocytopenic purpura (iTTP) is a rare autoimmune disorder characterized by severely reduced activity of the von Willebrand factor (VWF)-cleaving protease ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) due to autoantibodies. This leads to the development of pathogenic multimers of VWF, causing a thrombotic microangiopathy with decreased number of platelets, hemolysis, and life-threatening tissue ischemia of mostly brain, heart, and kidneys. Standard treatment of iTTP involves daily plasma exchange to remove ultra large multimers of VWF, inhibitors, substituting ADAMTS13, and the accompaniment of an immunosuppressive treatment with steroids. Recently, caplacizumab was approved for iTTP. Caplacizumab is a nanobody binding the A1 domain of VWF, blocking its interaction with glycoprotein Ib-IX-V platelet receptor and therefore preventing platelet aggregation. VWF activities may serve as therapeutic drug monitoring of caplacizumab, whereas ADAMTS13 activities may be used for biomarkers to guide caplacizumab treatment modalities and overall treatment duration. Additional immunosuppressive treatment by inhibiting autoantibody formation (e.g., the use of Rituximab, a chimeric monoclonal antibody directed against the B-cell antigen CD20) is a further treatment option. Infections are well-known causes for an acute episode for patients with iTTP. The novel SARS-CoV-2 virus is mainly associated with acute respiratory distress as well as diffuse endothelial inflammation and increased coagulopathy. However, little is known about an infection with SARS-CoV-2 virus triggering iTTP relapses. We herein report the case of an acute iTTP episode accompanying a SARS-CoV-2 infection.
Subject(s)
COVID-19 , Purpura, Thrombocytopenic, Idiopathic , Purpura, Thrombotic Thrombocytopenic , Thrombosis , Humans , Purpura, Thrombotic Thrombocytopenic/diagnosis , Purpura, Thrombotic Thrombocytopenic/therapy , von Willebrand Factor/metabolism , COVID-19/complications , SARS-CoV-2 , Purpura, Thrombocytopenic, Idiopathic/diagnosis , Purpura, Thrombocytopenic, Idiopathic/drug therapy , Immunosuppressive Agents/therapeutic use , Thrombosis/drug therapy , Autoantibodies , ADAMTS13 Protein/therapeutic useABSTRACT
PURPOSE: Informed consent is required prior to any medical procedure. In the context of cancer treatment, special efforts are needed to inform cancer patients properly about treatment, potential sequelae and alternative therapies. Little is known about the effectiveness of current informed consent strategies and patients' individual satisfaction. Given the heterogeneity in terms of age, education, sex and other factors, detailed understanding of patients' comprehension and perception is the basis for further optimization of the informed consent process, which was the aim of the current investigation. METHODS: Patients with a new cancer diagnosis and recent informed consent were asked to complete a questionnaire about satisfaction, comprehension, time management, physician-patient relationship and other items of the informed consent process. Patients were followed for 6 months and invited to complete a follow-up questionnaire. RESULTS: In total, 89 patients completed the first questionnaire and 52 the follow-up questionnaire. Subjective understanding was assumed high, however, this did not correlate with objective understanding. Age and education were identified as influencing factors for comprehension. 85% of the patients were satisfied with the information provided. A major gap was the information on alternative therapies. Moreover, not all patients perceived the consent dialog as such, and particularly the individual treatment intention partially remained unclear for some patients. CONCLUSIONS: To ensure that informed consent is based on solid understanding, informed consenting must be patient-centered and consider the individual expectations, needs and abilities of cancer patients. Further studies are required to develop tailored informed consent strategies.
