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Diagnose: Thrombotisch thrombozytopenische Purpura (TTP)

Diagnosis TTP

Thrombotic thrombocytopenic purpura (medical abbreviation TTP), also known as aTTP (acquired thrombotic thrombocytopenic purpura), iTTP (immune-mediated thrombotic thrombocytopenic purpura) or Moschcowitz syndrome, is a chronic autoimmune disease. Very rarely it is caused by a hereditary genetic defect.

In TTP, blood clots form in many parts in the body, which then block the smallest vessels in the organs and can lead to a variety of symptoms (e.g. skin haemorrhages, fever, malaise, neurological symptoms). This is caused by reduced activity of the enzyme ADAMTS13. If left untreated, TTP is associated with a significantly reduced life expectancy and quality of life. Cardiovascular events, in particular heart attacks and strokes, being the main reason. Those affected also have an increased risk of vascular dementia and depression.

Have you been diagnosed with TTP? Do you have questions about the treatment of TTP? Our team of doctors will be happy to answer your personal questions. Please use the contact form.

FOCUS ON TTP COLOGNE UNIVERSITY HOSPITAL

TTP is mostly, an autoimmune disease which is fatal in over 90% of cases if left untreated. Recurrences are common and affect more than 30-50% of all patients. At the University Hospital Cologne, we have set up specialised consultation hours for TTP and other autoimmune diseases. You can find further information as well as contact details for making an appointment at our consultation centre here.

If you suspect an acute flare-up or have unclear symptoms of the disease, please come and see us at our Central Emergency Room.

REACT-2020-REGISTER //
TTP PATIENT REGISTRY

With caplacizumab (Cablivi®), a new drug for the treatment of TTP has been available in Germany since 2018, which is approved in combination with plasma separation (blood plasma is removed from the body and exchanged for donor plasma) and immunosuppression. We have established the REACT 2020 registry in order to better understand the disease and to be able to precisely control the new forms of therapy with caplacizumab on an individual basis. In this observational study, we are gathering experience from as many TTP patients as possible. By participating in the registry (voluntarily, of course), you can actively help to improve the treatment options for TTP. If you are interested, please use our contact form.

Participating centers - experts in your area

TTP RESEARCH AT THE
UNIKLINIK KÖLN

Our TTP study group is continuously working to improve the diagnosis and treatment of TTP. In close cooperation with our German and international partners, we have already been able to show through the REACT 2020 registry that caplacizumab can be used to treat selected cases without invasive plasma separation and that the intensity of therapy can be reduced during the course of an acute TTP relapse.

Publications

Völker LA, Kaufeld J, Balduin G, Merkel L, Kühne L, Eichenauer DA, Osterholt T, Hägele H, Kann M, Grundmann F, Kolbrink B, Schulte K, Gäckler A, Kribben A, Boss K, Potthoff SA, Rump LC, Schmidt T, Mühlfeld AS, Schulmann K, Hermann M, Gaedeke J, Sauerland K, Bramstedt J, Hinkel UP, Miesbach W, Bauer F, Westhoff TH, Bruck H, Buxhofer-Ausch V, Müller TJ, Wendt R, Harth A, Schreiber A, Seelow E, Tölle M, Gohlisch C, Bieringer M, Geuther G, Jabs WJ, Fischereder M, von Bergwelt-Baildon A, Schönermarck U, Knoebl P, Menne J, Brinkkoetter PT; German TTP-Study Group. Impact of first-line use of caplacizumab on treatment outcomes in immune thrombotic thrombocytopenic purpura - PubMed (nih.gov). J Thromb Haemost. 2022 Dec 22:S1538-7836(22)07624-3.

Eller K, Knoebl P, Bakkaloglu SA, Menne JJ, Brinkkoetter PT, Grandt L, Thiem U, Coppo P, Scully M, Haller MC. European Renal Best Practice endorsement of guidelines for diagnosis and therapy of thrombotic thrombocytopaenic purpura published by the International Society on Thrombosis and Haemostasis - PubMed (nih.gov). published by the International Society on Thrombosis and Haemostasis. Nephrol Dial Transplant. 2022 Jun 23;37(7):1229-1234.

Kühne L, Kaufeld J, Völker LA, Wendt R, Schönermarck U, Hägele H, Osterholt T, Eichenauer DA, Bieringer M, von Bergwelt-Baildon A, Fischereder M, Buxhofer-Ausch V, Menne J, Brinkkoetter PT, Knöbl P. Alternate-day dosing of caplacizumab for immune-mediated thrombotic thrombocytopenic purpura - PubMed (nih.gov). Alternate-day dosing of caplacizumab for immune-mediated thrombotic thrombocytopenic purpura. J Thromb Haemost. 2022 Apr;20(4):951-960.

Kühne L, Völker LA, Hagmann H, Hägele H, Osterholt T, Eichenauer DA, Thomas A, Breuer J, Grüttner B, Gottschalk I, Kann M, Benzing T, Thevis M, Müller AM, Brinkkoetter PT. First use of the anti-VWF nanobody caplacizumab to treat iTTP in pregnancy - PubMed (nih.gov). Br J Haematol. 2022 Feb;196(3):e30-e33.

Kaufeld J, Brinkkoetter PT, Schreiber A, Jabs WJ, Bieringer M, Bruck H, Menne J, Völker LA. Caplacizumab: frequent local skin reactions - PubMed (nih.gov). Ann Hematol. 2021 Dec;100(12):3051-3052.

Völker LA, Brinkkoetter PT, Knöbl PN, Krstic M, Kaufeld J, Menne J, Buxhofer-Ausch V, Miesbach W. Treatment of acquired thrombotic thrombocytopenic purpura without plasma exchange in selected patients under caplacizumab - PubMed (nih.gov). J Thromb Haemost. 2020 Nov;18(11):3061-3066.

Völker LA, Kaufeld J, Miesbach W, Brähler S, Reinhardt M, Kühne L, Mühlfeld A, Schreiber A, Gaedeke J, Tölle M, Jabs WJ, Özcan F, Markau S, Girndt M, Bauer F, Westhoff TH, Felten H, Hausberg M, Brand M, Gerth J, Bieringer M, Bommer M, Zschiedrich S, Schneider J, Elitok S, Gawlik A, Gäckler A, Kribben A, Schwenger V, Schoenermarck U, Roeder M, Radermacher J, Bramstedt J, Morgner A, Herbst R, Harth A, Potthoff SA, von Auer C, Wendt R, Christ H, Brinkkoetter PT, Menne J. ADAMTS13 and VWF activities guide individualized caplacizumab treatment in patients with aTTP - PubMed (nih.gov). Blood Adv. 2020 Jul 14;4(13):3093-3101.

Völker LA, Kaufeld J, Miesbach W, Brähler S, Reinhardt M, Kühne L, Mühlfeld A, Schreiber A, Gaedeke J, Tölle M, Jabs WJ, Özcan F, Markau S, Girndt M, Bauer F, Westhoff TH, Felten H, Hausberg M, Brand M, Gerth J, Bieringer M, Bommer M, Zschiedrich S, Schneider J, Elitok S, Gawlik A, Gäckler A, Kribben A, Schwenger V, Schoenermarck U, Roeder M, Radermacher J, Bramstedt J, Morgner A, Herbst R, Harth A, Potthoff SA, von Auer C, Wendt R, Christ H, Brinkkoetter PT, Menne J. Real-world data confirm the effectiveness of caplacizumab in acquired thrombotic thrombocytopenic purpura - PubMed (nih.gov). Blood Adv. 2020 Jul 14;4(13):3085-3092.

Völker LA, Grundmann F, Brinkkötter PT. [Acquired thrombotic-thrombocytopenic purpura - patient management with the advent of novel therapeutic agents] - PubMed (nih.gov) Dtsch Med Wochenschr. 2019 Nov;144(22):1572-1575. German.

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Nephrologisches Forschungslabor
Universitätsklinik Köln
CECAD Research Center
Joseph-Stelzmann-Str. 26
50931 Köln
Tel. +49 221 / 478 - 89030
Fax +49 221 / 478 - 148 9680
nephrolab@kidneyresearchcenter.org