Prevention of relapse in patients with acquired thrombotic thrombocytopenic purpura undergoing elective surgery: a case series
Author(s): ,
Sara Arcudi
Affiliations:
Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy
,
Barbara Ferrari
Affiliations:
Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy
,
Silvia Pontiggia
Affiliations:
Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy
,
Antonella Tufano
Affiliations:
Department of Clinical Medicine and Surgery, AOU Federico II, Naples, Italy
,
A. Artoni
Affiliations:
Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy
,
Illaria Mancini
Affiliations:
Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy
Flora Peyvandi
Affiliations:
Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy. Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy
Correspondence: Flora Peyvandi, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center ‐ Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, UOC Medicina Generale ‐ Emostasi e Trom
ISTH Academy. Peyvandi F. Mar 1, 2019; 273410
Dr. Flora Peyvandi
Dr. Flora Peyvandi
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Background
Severe ADAMTS‐13 deficiency has been recognized as the main risk factor for recurrence of thrombotic thrombocytopenic purpura (TTP). Several conditions, including surgery, may influence the levels of ultra‐large von Willebrand factor and ADAMTS‐13, acting as a trigger for an acute TTP event.
Objectives
To report our experience of management of six patients with acquired TTP who underwent elective surgery after prophylactic treatment to restore ADAMTS‐13 activity levels.
Patients
Six patients followed for acquired TTP with severe ADAMTS‐13 deficiency during remission were candidates for seven elective surgeries (inguinal hernioplasty, cholecystectomy, laparoscopic hysterectomy, oophorectomy, parotidectomy and two total hip arthroplasties).
Results
Four patients were treated with prophylactic plasma exchange (PEX) therapy immediately before surgery. One patient was treated with PEX therapy before her first surgery and with preemptive rituximab once her second surgery was scheduled. Because rituximab increased ADAMTS‐13 levels only partially, she required one PEX procedure the day before her second surgery. One patient was treated with azathioprine after rituximab failure, obtaining a progressive increase of ADAMTS‐13 activity to more than 40%. This level allowed her to undergo total hip arthroplasty without additional treatment. All surgeries were successful and no complications or relapses occurred.
Conclusions
Six patients with acquired TTP underwent seven successful surgical procedures using prophylaxis to restore ADAMTS‐13 activity. Further observational studies or randomized clinical trials are needed to confirm whether prophylactic PEX could be the key factor in preventing relapse.
Keyword(s)
ADAMTS‐13 protein, general surgery, plasmapheresis, purpura, thrombotic thrombocytopenic, von Willebrand factor
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