Postsurgical thrombotic microangiopathy: Case series and review of the literature

Abstract

Objectives Describe the clinical presentation, treatment, and outcomes of postsurgical thrombotic microangiopathy (TMA). Methods In this retrospective study, records of individuals diagnosed with TMA developing within 30 days of a surgical procedure at Mayo Clinic from 2000 to 2016 were reviewed. Available literature regarding postsurgical TMA was comparatively reviewed. Results Twenty patients were diagnosed with TMA developing within 30 (median 6.5, range (1‐28)) days) following a procedure. Preceding procedures included orthopedic (n = 4), vascular (n = 4), abdominal (n = 8), thoracic (n = 2), and other (n = 2). Review of the literature identified 65 patients with postsurgical TMA and cardiovascular procedures were the most common preceding surgery. The majority of patients in the current cohort and literature were treated with therapeutic plasma exchange (TPE). Among the evaluable patients in the current cohort, 100% demonstrated response to TPE; however, 25% required the addition of other therapy including eculizumab to maintain a response 80% of patients in the literature demonstrated a response to TPE. Conclusions Although rare, early recognition and treatment of postsurgical TMA can lead to good outcomes. More research is necessary to determine the underlying pathophysiology and optimal treatment for postsurgical TMA.

Prospective Evaluation of the Pre-, Intra-, and Postoperative Kinetics of ADAMTS-13, von Willebrand Factor, and Interleukin-6 in Vascular Surgery

“…It has not yet been established why surgery-associated TTP resembling classical TTP can occur even though ADAMTS-13 activity is only moderately reduced. Nor has the clinical relevance of mildly reduced intraoperative and postoperative ADAMTS-13 activity been clarified 24 ; no patient in this study showed clinical appearance of TTP. These findings may suggest that the level of ADAMTS-13 activity is not the only essential factor in the development of surgery-associated TMA.…”

“… 14 Recent investigations could not detect any appearance of new inhibitory ADAMTS-13 antibodies after cardiac surgery. 6 In contrast to these findings, Sridharan et al 24 identified 2 of the patients in their institutional review with immune-mediated pTTP. In both reviews, cardiovascular and vascular surgery were identified as the most common procedures leading to pTTP.…”

“…The finding that most patients with pTTP described in the literature responded to plasma exchange may support the hypothesis that plasmatic imbalance is responsible for surgery-associated TMA. 24 Systemic inflammation-as potentially caused by major surgery-was shown to be associated with increased ULVWF and decreased ADAMTS-13 levels. 36,38 Investigating intensive care patients in inflammatory and septic states, Bockmeyer et al 39 found that acquired ADAMTS-13 deficiency was associated with the severity of inflammatory host response regardless of the underlying mechanism and an imbalance between levels of ADAMTS-13 and its substrate that was associated with the appearance of ULVWF multimers in the circulation.…”

“…The finding that most patients with pTTP described in the literature responded to plasma exchange may support the hypothesis that plasmatic imbalance is responsible for surgery-associated TMA. 24 …”

“…Postoperative TTP is a rare but life-threatening disease. As only a limited number of case reports on pTTP exists (one current review article by Sridharan et al 24 included 85 cases), indicating an incidence would be speculative, especially since the number of unrecognized cases might be significantly higher than the amount of cases correctly diagnosed and mentioned in literature. Common postoperative complications such as blood loss, infections, or heparin-induced thrombocytopenia present with a similar clinical picture and can delay or obscure the correct diagnosis.…”