Plasma exchange in thrombotic thrombocytopenic purpuras

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Published May 30, 2024
https://doi.org/10.62438/tunismed.v102i6.4614
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Vol. 102 No. 6 (2024): Tunis med jun 2024

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Dorra Jabr
Clinical Haematology Department, Aziza Othmana Hospital, Faculty of Medicine of Tunis, University of Tunis el Manar, Tunis, Tunisia
Roua Hsasna
Clinical Haematology Department, Aziza Othmana Hospital, Faculty of Medicine of Tunis, University of Tunis el Manar, Tunis, Tunisia
Salma Kéfi
Clinical Haematology Department, Aziza Othmana Hospital, Faculty of Medicine of Tunis, University of Tunis el Manar, Tunis, Tunisia
Rachid Kharrat
Clinical Haematology Department, Aziza Othmana Hospital, Faculty of Medicine of Tunis, University of Tunis el Manar, Tunis, Tunisia
Hend Ben Néji
Clinical Haematology Department, Aziza Othmana Hospital, Faculty of Medicine of Tunis, University of Tunis el Manar, Tunis, Tunisia
Raihane Ben Lakhal
Clinical Haematology Department, Aziza Othmana Hospital, Faculty of Medicine of Tunis, University of Tunis el Manar, Tunis, Tunisia

Abstract

Introduction : Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially fatal hematological disorder that requires urgent treatment. Once the diagnosis has been made, plasma exchange (PE) must be started immediately and until a response is obtained.


 Aim : Evaluate PE in terms of responses and complications in the treatment of TTP.


Methods : This was a monocentric, descriptive, retrospective study including patients in whom TTP was diagnosed and treated with plasmapheresis in the clinical hematology department at Aziza Othmana Hospital, between January 2010 and December 2020.


Results : Our study included 26 patients. PE was initiated within a median of 1 day. The rhythm of exchanges was daily in 22 patients. Twenty PE-related complications were noted, hypocalcemia being the most frequent (30%). CR was achieved in 15 patients after PE alone. Nine patients were refractory, and six received 2nd-line treatment, with CR achieved in five patients. Relapse was noted in six patients (40%). They were treated by PE and only one patient received rituximab. Four patients had a response. The overall response rate was 69% and overall mortality was 30%. OS at 2 years was 68,3% and RFS was 56,5% Factors associated with the achievement of CR were the fall in LDH at D5 of treatment (p=0,027,OR=0,59 ;IC 95%[0,32-1,08])  and the daily rhythm of PE  (p=0,005, OR=0,35; IC 95%[0,14-0,91]).


Conclusion : Our results were comparable to those of the literature, but the rate of refractory disease was higher. Rituximab may enhance our results.

Keywords:

Thrombotic thrombocytopenic purpura, Thrombotic microangiopathies , Plasmapheresis, Survival

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