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.