Long‐term outcomes of thrombotic microangiopathy treated with plasma exchange: A systematic review

Abstract

With the adoption of plasma exchange as standard treatment for thrombotic microangiopathy (TMA), more patients are surviving and long-term outcomes have greater relevance. We conducted a systematic review to synthesize and evaluate the quality of evidence on long-term outcomes of TMA among adults treated with plasma exchange and to identify factors that may be associated with a worse long-term prognosis. We searched databases from 1980 to 2013 for eligible articles published in any language. We included studies that reported outcomes in at least ten adults with a history of TMA treated with plasma exchange and at least 6 months of follow-up. We abstracted data in duplicate and assessed the methodological quality of each study using an assessment tool developed based on recommended validity criteria. We screened 6672 articles, reviewed 213, and included 34 studies totaling 1182 patients (study median [range], 24 ). The mean (or median) follow-up ranged from 6 months to 13 years. The cumulative incidence of relapse and mortality was highly variable and ranged from 3 to 84 and 0 to 61%, respectively. The incidence of other outcomes across 10 studies also varied (outcomes included hypertension, kidney disease, preeclampsia, stroke, seizure, severe cognitive impairment, and depression); in three other studies, long-term neurocognitive function and health-related quality of life were significantly lower than in the general population. Patients who survive an episode of TMA may be susceptible to long-term vascular complications, but the magnitude of this risk and how to mitigate it remains unclear.

Does morphology matter in 2017? An approach to morphologic clues in non‐neoplastic blood and bone marrow disorders

“…Plasma exchange has also been used in other types of MAHA with varying success. Without treatment, patients with thrombotic microangiopathy progress to kidney failure, neurologic deterioration, cardiac injury, and death in 90% of cases …”

Cost analysis of the impact of caplacizumab in the treatment of acquired thrombotic thrombocytopenic purpura from a US hospital perspective

“…The reported incidence for this condition is 1.2-11 cases per million (4-5 cases per million in the US) [2][3][4][5] , and it mainly affects young and otherwise healthy adults aged 30-50 years 6 . It is caused by inhibitory autoantibodies to von Willebrand factor-cleaving enzyme ADAMTS13 [7][8][9] ; severely decreased ADAMTS13 activity leads to the extensive uncontrolled formation of microvascular thrombi, which can result in tissue ischemia, organ dysfunction, and/or death [10][11][12][13][14][15][16] . aTTP has been treated for many years with therapeutic plasma exchange (TPE) and immunosuppressive therapy, and a considerable proportion of patients are refractory to therapy and/or experience multiple exacerbations/ relapses 11,[17][18][19][20] .…”

Postsurgical thrombotic microangiopathy: Case series and review of the literature

“…1 TMAs can be fatal if early diagnosis with initiation of treatment is not undertaken. 2,3 Not only is early recognition of a TMA necessary for preventing fatal outcomes but clinical differentiation between the various diagnoses is also essential as that will dictate what treatment ensues.…”

“…Although the syndrome has characteristic clinical and pathological features, the etiology of each disorder is different and include acquired triggers and inherited predispositions . TMAs can be fatal if early diagnosis with initiation of treatment is not undertaken . Not only is early recognition of a TMA necessary for preventing fatal outcomes but clinical differentiation between the various diagnoses is also essential as that will dictate what treatment ensues.…”