EHA Library - The official digital education library of European Hematology Association (EHA)

The data was collected from year 2012 to 2016 from Ampang Hospital via the electronic hospital information system (EHIS) and external records traced from Haemostasis laboratory in Ampang Hospital as well as from other hospitals nationwide.

There were total of 243 suspected TMA cases, encompassing 97 (39.9%) males and 146 (60.1%) females. The median age for this cohort was 34 years. Only 54 (24.15%) patients were diagnosed as TTP based on ADAMTS-13 activity ≤10%. Treatments were evaluated by using complete case details from Ampang Hospital cohort (69 cases). From this cohort, only 59 cases had ADAMTS-13 activity testing. There were 24% Primary Acquired TTP, 5% typical HUS, 3.4% atypical HUS, 3.4% Pregnancy TMA, 3.4% SLE related TMA, 20.3% Transplant TMA, 1.7% Cancer associated TMA and 37% TMA of other causes. The average plasma exchange was 8.4 cycles, and was higher in patients with ADAMTS-13 activity of ≤10% (11.4 cycles) as compared to those with ADAMTS-13 > 10% (7.7 cycles). No infectious diseases were transmitted as a result of plasma exchange or plasma infusion. Treatments used in the patients included immunosuppressant therapy like methylprednisolone (85.5%), monoclonal antibody like rituximab (36.2%), bortezomib (11.6%), cyclophosphamide (10.1%), cyclosporine (10.1%), and vincristine (26.1%). The survival outcome seemed to be worse among the transplant TMA in comparison to other groups (log-rank, p<0.0001). Transplantation was also associated with higher odd of death among TMA cases (OR: 14.8571, 95% CL: 1.7385, 126.9707). Those with confirmed TTP was inevitably doing better than the others in terms of overall survival (log-rank, p=0.0299). The odds of death was 4.36 times higher in patients with ADAMTS-13 activity > 10% (OR: 4.36, 95% CL: 1.0961, 17.3714), indicating secondary TTP may have inferior treatment and disease outcomes than primary TTP like congenital or acquired TTP. Besides, the complications of the disease were also evaluated which revealed 26.9% of renal failure and 52.2% of neurological deficit. Furthermore, 8.7% were complicated by Venous Thromboembolism, either provoked or spontaneous. The odds of relapse is 2.9 times higher given the ADAMTS-13 activity ≤10% to ADAMTS-13 activity >10%.