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Eur J Haematol. 2016 Aug;97(2):183-91. doi: 10.1111/ejh.12706. Epub 2016 Jan 6.

Twice-daily therapeutical plasma exchange-based salvage therapy in severe autoimmune thrombotic thrombocytopenic purpura: the French TMA Reference Center experience.

Soucemarianadin M1,2, Benhamou Y1,3,4, Delmas Y1,5, Pichereau C6,7, Maury E1,6,7, Pène F1,8,9, Halimi JM1,10,11, Presne C1,12, Thouret JM2, Veyradier A1,13,14, Coppo P1,6,15,16.

Author information

1
Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Paris, France.
2
Service de Réanimation, Centre Hospitalier Métropole Savoie, Chambéry, France.
3
Inserm U1096, Rouen, France.
4
Service de Médecine Interne, CHU Charles Nicolle, Rouen, France.
5
Service de Néphrologie Transplantation Dialyse, Centre Hospitalier Universitaire, Bordeaux, France.
6
Sorbonne Université, UPMC Univ Paris 06, Paris, France.
7
Service de Réanimation Médicale, CHU Saint-Antoine, Paris, France.
8
Service de Réanimation Polyvalente, Hôpital Cochin, Paris, France.
9
Université Paris 5, Paris, France.
10
Service de néphrologie-immunologie clinique, hôpital Bretonneau, Tours, France.
11
EA 4245, université François-Rabelais 2, boulevard Tonnellé, Tours, France.
12
Service de Néphrologie - Médecine Interne, Hôpital Sud, Amiens, France.
13
Service d'Hématologie Biologique, Hôpital Lariboisière, Paris, France.
14
Univ. Paris Diderot, Sorbonne Paris Cité, Paris, France.
15
Inserm U1009, Institut Gustave Roussy, Villejuif, France.
16
Service d'Hématologie, Hôpital Saint-Antoine, Paris, France.

Abstract

BACKGROUND:

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.

METHODS:

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.

RESULTS:

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).

CONCLUSIONS:

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.

KEYWORDS:

ADAMTS13; autoimmune thrombotic thrombocytopenic purpura; twice-daily therapeutic plasma exchange

PMID:
26608617
DOI:
10.1111/ejh.12706
[Indexed for MEDLINE]
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