We were delighted by the great interest in our review article on the differential diagnosis and treatment of thrombotic microangiopathies (1). The multitude of potentially involved specialty disciplines in particular presents huge challenges in terms of the appropriate explanation of relevant differential diagnoses and differential therapeutic measures. Hassenpflug and Schneppenheim name several aspects that distinguish thrombotic-thrombocytic purpura (TTP) in children and adolescents from TTP in adults:
- An early diagnosis of hereditary TTP by means of determining ADAMTS13 activity in unclear findings of hemolysis/thrombopenia in neonates makes sense as in children and adolescents, about one-third of all cases of TTP are due to an ADAMTS13 mutation. By contrast, the probability for hereditary TTP in adults is notably below 5% (2).
- The appropriate treatment in case of active TTP in patients with known congenital TTP can consist merely of fresh plasma substitution. In these cases, the—extremely laborious—plasmapheresis is not necessary. Testing for ADAMTS13 activity/antigen and ADAMTS13 antibodies in order to distinguish between hereditary and acquired TTP with an analysis period of a few hours is desirable but not (yet) available in all hospitals. Since acquired, antibody mediated TTP is much more common (>95%) especially in adulthood, plasmapheresis is still recommended in adult cases of unclear etiology.
On behalf of the authors
PD Dr. med. Martin Bommer
Klinik für Hämatologie, Onkologie, Palliativmedizin und Infektionskrankheiten,
Alb-Fils-Kliniken, Göppingen, firstname.lastname@example.org
Conflict of interest statement
Dr Bommer has received consultant honoraria and study support (third-party funds) for clinical studies from Ablynx and speaking honoraria from Alexion Pharmaceuticals, Sanofi Genzyme, and JAZZ Pharmaceuticals.
|1.||Bommer M, Wölfle-Guter M, Bohl S, Kuchenbauer F: The differential diagnosis and treatment of thrombotic microangiopathies. Dtsch Arztebl Int 2018; 115: 327–34 VOLLTEXT|
|2.||Joly BS, Coppo P, Veyradier A: Thrombotic thrombocytopenic purpura. Blood 2017; 129: 2836–46 CrossRef MEDLINE|