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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

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