Plasma exchange in thrombotic thrombocytopenic purpuras.

Autor:	Jabr D; Clinical Hematology Department, Aziza Othmana Hospital, Faculty of Medicine of Tunis, University of Tunis el Manar, Tunis, Tunisia., Hsasna R; Clinical Hematology Department, Aziza Othmana Hospital, Faculty of Medicine of Tunis, University of Tunis el Manar, Tunis, Tunisia., Kefi S; Clinical Hematology Department, Aziza Othmana Hospital, Faculty of Medicine of Tunis, University of Tunis el Manar, Tunis, Tunisia., Kharrat R; Clinical Hematology Department, Aziza Othmana Hospital, Faculty of Medicine of Tunis, University of Tunis el Manar, Tunis, Tunisia., Ben Neji H; Clinical Hematology Department, Aziza Othmana Hospital, Faculty of Medicine of Tunis, University of Tunis el Manar, Tunis, Tunisia., Ben Lakhal R; Clinical Hematology Department, Aziza Othmana Hospital, Faculty of Medicine of Tunis, University of Tunis el Manar, Tunis, Tunisia.
Jazyk:	francouzština
Zdroj:	La Tunisie medicale [Tunis Med] 2024 Jun 05; Vol. 102 (6), pp. 343-347. Date of Electronic Publication: 2024 Jun 05.
DOI:	10.62438/tunismed.v102i6.4614
Abstrakt:	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 84,4%. 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.
Databáze:	MEDLINE
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