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Preoperative prophylaxis may prevent relapse in acquired thrombotic thrombocytopenic purpura
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Prophylactic therapies restored ADAMTS13 activity levels prior to elective surgeries in six patients with acquired thrombotic thrombocytopenic purpura, according to results of an observational study published in Journal of Thrombosis and Haemostasis.
The surgeries all were successful, with no resulting relapses or complications, according to the researchers.
“Persistent severe ADAMTS13 deficiency during remission is recognized as the main risk factor for TTP [thrombotic thrombocytopenic purpura] recurrence,” Sara Arcudi, MD, and colleagues wrote. “Surgery, as well, has been described as a potential trigger for TTP, likely due to the direct damage of the microvasculature with subsequent release of [ultra-large von Willebrand factor] multimers in the bloodstream overwhelming ADAMTS13 activity.”
Researchers analyzed the results of therapeutic approaches to increase ADAMTS13 levels prior to elective surgery among six patients with acquired TTP and severe and persistent ADAMTS13 deficiency during remission.
Five of the six patients had previous chronic relapsing TTP.
Surgeries included parotidectomy, total hip arthroplasty, inguinal hernioplasty, cholecystectomy, laparoscopic hysterectomy and oophorectomy.
The first patient, a woman aged 65 years, had five episodes of TTP before undergoing a cholecystectomy and total hip arthroplasty. The woman received 3 consecutive days of plasma exchange therapy before her first surgery, which increased ADAMTS13 activity from less than 3% to 38%. She experienced no postoperative complications or relapse. Pre-emptive rituximab (Rituxan; Genentech, Biogen) 2 years later only partially restored ADAMTS13 levels, which increased from 24% to 45% after treatment with prophylactic plasma exchange therapy immediately before her second surgery.
Since the surgeries, the patient woman has had two more incidences of TTP relapses and was treated with prophylactic plasma exchange therapy, steroids, and rituximab (Rituxan; Genentech, Biogen) in both cases.
The second patient, a man aged 68 years, had five episodes of TTP before undergoing left inguinal hernioplasty. The man underwent plasma exchange therapy immediately before surgery, when his ADAMTS13 activity was undetectable, and demonstrated an increase in ADAMTS13 levels up to 25% after surgery.
Two years later, the he patient man experienced two TTP relapses and continues to have undetectable ADAMTS13 levels post-surgery and has had two incidences of TTP. He undergoes regular prophylactic plasma exchange therapy to manage the condition.
The third patient, a woman aged 58 years, had four episodes of TTP before undergoing a total laparoscopic hysterectomy. She received plasma exchange therapy the morning of her surgery because she had severely deficient ADAMTS13 activity (3%). The woman’s levels of ADAMTS13 increased to 50% and she has not had an incidence of TPP since surgery.
The fourth patient, a woman aged 49 years, had three TTP episodes before undergoing a laparoscopic oophorectomy. Her ADAMTS13 levels increased to 34% after the two rounds of plasma exchange therapy just before the surgery. She has had no incidences of TTP since the surgery.
The fifth patient, a woman aged 43 years, had seven TTP episodes before undergoing a total hip arthroplasty. She was treated with plasma exchange therapy, steroids and rituximab for six of the incidences, but was switched to 100 mg daily azathioprine after the seventh incidence.
Her ADAMTS13 levels increased to 42% before the surgery and were maintained at 33% following the surgery.
The sixth patient, a man aged 38 years, had one episode of TTP before undergoing a parotidectomy. The TTP event was treated with plasma exchange therapy and steroids, but he still had undetectable levels of ADAMTS13 activity with high-titer anti-ADAMTS13 antibodies.
One round of plasma exchange therapy right before surgery led to activity levels of up to 28% and a disappearance of anti-ADAMTS13 antibodies.
A lack of standardization in how patients should be treated before surgery served as a limitation to this study.
Randomized trials are needed to confirm the study’s results, according to Arcudi and colleagues.
“In conclusion we report seven cases of successful elective surgical procedures in six patients affected with acquired TTP with severe ADAMTS13 deficiency at remission,” Arcudi and colleagues wrote. “Our clinical experience suggests that these patients can undergo elective surgery after achieving safer ADAMTS13 activity obtained with prophylactic preoperative [plasma exchange therapy], especially in those cases unresponsive or not eligible for immunosuppression.” – by John DeRosier
Disclosures: Arcudi reports no relevant financial disclosures. One researcher reports honoraria from and an advisory board role with Ablynx.
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