3.3 Treatment and Outcomes
The dose of CNI was reduced once TA-TMA was formally diagnosed. Nineteen patients were treated with low-molecular-weight heparin, 17 patients were treated with plasma exchange, and the median number of plasma exchanges was 4 (3–18). Rituximab was used in 12 patients with a median of 1.5 (1–3) administrations. Two patients were treated with eculizumab and one was treated with defibrotide.
Among the 20 patients, 11 responded well to treatment, with hemoglobin and platelets both increasing, LDH levels and blood pressure decreasing, and no new ecchymosis or petechiae on the skin. Nine patients had a poor response to treatment and unfortunately died (45% mortality from diagnosis). The median time of death was 16 (5–77) days after diagnosis, and the main cause of death was disseminated intravascular coagulation and multi-organ failure. Of the 11 patients who were effectively treated, 4 eventually died of sepsis and acute respiratory failure within 136, 177, 279, and 602 days after diagnosis, respectively. Only seven patients survived to the end of the follow-up period and were reported to be healthy.