Treatment of acquired thrombotic thrombocytopenic purpura without plasma exchange in selected patients under caplacizumab.
J Thromb Haemost
; 18(11): 3061-3066, 2020 11.
Article
em En
| MEDLINE
| ID: mdl-32757435
ABSTRACT
BACKGROUND:
Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare, life-threatening autoimmune thrombotic microangiopathy. Current standard of care is therapeutic plasma exchange, immunosuppression, and caplacizumab, an anti-von Willebrand factor nanobody, which is effective in treating aTTP episodes. PATIENTS/METHODS:
Here we report on seven episodes of aTTP treated without plasma exchange in six female patients in Germany and Austria. Two episodes were initial presentations of aTTP; in five instances, patients experienced a relapse. In four episodes, moderate to severe organ dysfunction was observed; three cases presented with a mild course. All patients received caplacizumab immediately once aTTP was suspected or diagnosed, and plasma exchange was omitted based on shared decision making between patient and the treating physicians.RESULTS:
We observed a rapid and robust increase of platelet counts already after the first dose of caplacizumab, leading to a doubling of platelet counts within 17 hours (median), platelet counts normalized (>150 G/L) after median 84 hours. Lactate dehydrogenase, as a surrogate parameter of organ damage, improved in parallel to the platelet counts, indicating resolving microangiopathy.CONCLUSIONS:
In conclusion, in selected cases of acute bouts of aTTP, it seems feasible to delay or omit plasma exchange if platelet counts increase and organ function is stable after start of caplacizumab therapy.Palavras-chave
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Base de dados:
MEDLINE
Assunto principal:
Púrpura Trombocitopênica Trombótica
Idioma:
En
Ano de publicação:
2020
Tipo de documento:
Article