Long term outcome and sequelae in patients after acute thrombotic thrombocytopenic purpura episodes^[*]

Summary

We report on 21 patients with idiopathic thrombotic thrombocytopenic purpura (TTP) whose courses of disease have been followed from the respective diagnosis until now. They had a documented ADAMTS13 activity below 5%, a high autoantibody titer and detectable ultralarge von Willebrand factor (VWF) multimers during their episodes. The initial diagnosis was based on clinical symptoms and on laboratory parameters: thrombocytopenia, haemolytic anaemia, schistocytes and an increased LDH level. 103 acute clinical episodes of 21 TTPpatients during a time period of 30 years are described. Case histories, comorbidities and sequelae were retrospectively documented. Results, conclusion: Although patients are consistently in a prothrombotic status, clinical acute manifestations only occur after triggering. Most common trigger factors are gastrointestinal infections and pregnancy. The relapse risk per month is 0.026; men have a higher risk for relapses (0.044) than women (0.021). Patients recover physically well, except for renal insufficiency in four cases. Nevertheless, major portion of patients suffers persistently from depression, anxiety disorders and persistent neurocognitive impairments.

Zusammenfassung

Wir berichten über 21 Patienten mit idiopatischer thrombotisch thrombozytopenischer Purpura (TTP), deren Krankheitsverlauf von der Diagnose bis heute verfolgt wurde. Die Patienten wiesen zum Zeitpunkt der Schübe eine ADAMTS13-Aktivität unter 5%, hohe Autoantikörpertiter und ultralange VWFMultimere auf. Die initiale Diagnose erfolgte aufgrund klinischer Symptome und Labordaten: Thrombozytopenie, hämolytische Anämie, Schistozyten und LDH. Insgesamt war-den 103 Schübe von 21 TTP-Patienten über einen Zeitraum von 30 Jahren beschrieben. Krankheitsverlauf sowie Begleiterkrankungen und Folgen der TTP wurden retrospektiv erfasst. Ergebnisse, Schlussfolgerungen: Obwohl die Patienten sich in einem dauerhaft prothrombotischen Status befinden, treten TTP-Schübe meist infolge zusätzlicher Auslöser, insbesondere gastrointestinaler Infektionen sowie Schwangerschaften, auf. Das Risiko eines Rückfalls des Kollektivs beträgt 0,026 pro Monat, wobei Männer ein höheres Rückfallrisiko aufweisen (0,044) als Frauen (0,021). Bis auf vier Patienten mit chronischer Niereninsuffizienz, erholten sich die Patienten körperlich gut. Dennoch leidet ein Großteil der Patienten unter Depressionen, Angststörungen und neurokognitiven Defiziten.

Keywords

Thrombotic thrombocytopenic purpura - ADAMTS13 activity - long term outcome - neurocognitive impairment

Schlüsselwörter

Thrombotisch thrombozytopenische Purpura - ADAMTS13 Aktivität - Spätfolgen - neuro -kognitive Störungen

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