Twice‐daily therapeutical plasma exchange‐based salvage therapy in severe autoimmune thrombotic thrombocytopenic purpura: the French TMA Reference Center experience
Daily therapeutic plasma exchange (TPE) and rituximab improved thrombotic thrombocytopenic purpura (TTP) prognosis. In the more severe cases, salvage therapies including twice-daily TPE and/or cyclophosphamide may be proposed and require evaluation. TTP was defined as a thrombotic microangiopathy (TMA) with severe (<10%) acquired ADAMTS13 deficiency. Among patients included in the French Reference Center for TMA registry, we considered those with a severe disease (i.e., unresponsive to daily TPE and rituximab) who received twice-daily TPE. Nineteen of 289 (6.6%) patients with TTP were treated by twice-daily TPE between 2008 and 2014. Twice-daily TPE was associated with rituximab in 16 cases. The median duration of twice-daily TPE treatment was 3 d (2-22 d). In 6 patients (31.6%), additional treatments (mainly pulses of cyclophosphamide) were performed because of a persistently refractory disease (4 cases) or an exacerbation (2 cases), despite twice-daily TPE. Only one patient (5.3%) died. The other 18 achieved a durable complete remission 25.5 d (13-68 d) after the first TPE. The median follow-up was 14.4 months (7 d-45 months). Twice-daily TPE may be an efficient strategy in the more severe TTP patients with a short-term life-threatening disease that could overcome their poor prognosis..
| Medienart: |
Artikel |
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| Erscheinungsjahr: |
2016 |
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| Erschienen: |
2016 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:97 |
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| Enthalten in: |
European journal of haematology - 97(2016), 2, Seite 183-191 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Soucemarianadin, Myriam [VerfasserIn] |
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| Links: |
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| BKL: | |
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| Themen: |
ADAMTS |
| doi: |
10.1111/ejh.12706 |
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| Förderinstitution / Projekttitel: |
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| 245 | 1 | 0 | |a Twice‐daily therapeutical plasma exchange‐based salvage therapy in severe autoimmune thrombotic thrombocytopenic purpura: the French TMA Reference Center experience |
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| 520 | |a Daily therapeutic plasma exchange (TPE) and rituximab improved thrombotic thrombocytopenic purpura (TTP) prognosis. In the more severe cases, salvage therapies including twice-daily TPE and/or cyclophosphamide may be proposed and require evaluation. TTP was defined as a thrombotic microangiopathy (TMA) with severe (<10%) acquired ADAMTS13 deficiency. Among patients included in the French Reference Center for TMA registry, we considered those with a severe disease (i.e., unresponsive to daily TPE and rituximab) who received twice-daily TPE. Nineteen of 289 (6.6%) patients with TTP were treated by twice-daily TPE between 2008 and 2014. Twice-daily TPE was associated with rituximab in 16 cases. The median duration of twice-daily TPE treatment was 3 d (2-22 d). In 6 patients (31.6%), additional treatments (mainly pulses of cyclophosphamide) were performed because of a persistently refractory disease (4 cases) or an exacerbation (2 cases), despite twice-daily TPE. Only one patient (5.3%) died. The other 18 achieved a durable complete remission 25.5 d (13-68 d) after the first TPE. The median follow-up was 14.4 months (7 d-45 months). Twice-daily TPE may be an efficient strategy in the more severe TTP patients with a short-term life-threatening disease that could overcome their poor prognosis. | ||
| 540 | |a Nutzungsrecht: © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd | ||
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| 700 | 1 | |a Delmas, Yahsou |4 oth | |
| 700 | 1 | |a Pichereau, Claire |4 oth | |
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| 700 | 1 | |a Pène, Frédéric |4 oth | |
| 700 | 1 | |a Halimi, Jean‐Michel |4 oth | |
| 700 | 1 | |a Presne, Claire |4 oth | |
| 700 | 1 | |a Thouret, Jean‐Marc |4 oth | |
| 700 | 1 | |a Veyradier, Agnès |4 oth | |
| 700 | 1 | |a Coppo, Paul |4 oth | |
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