Results
Total 73 transplants (51 allogeneic, 22 autologous) had been performed
in 63 patients during the study period. Six patients (4 males and 2
females) developed TA-TMA at a median duration of 2.5 months post-HSCT.
Incidence rate was 9.52%. For the six TA-TMA patients, five underwent
allogenic HSCT while one underwent autologous HSCT. Three out of six
cases of TA-TMA were histologically proven. Demographics and transplant
details are described in Table 1a . All five patients who
underwent allogeneic HSCT developed various degrees of acute
graft-versus-host disease (GVHD) and viral infections. Four used
calcineurin inhibitor (CNI) cyclosporine (CSA) as GVHD prophylaxis which
was stopped once TA-TMA was suspected. Three out of six patients
developed hepatic sinusoidal obstruction syndrome with defibrotide
given. Genetic predisposition had not been identified in local cohort
except identification of a variant of unknown significance in THBD gene
for Case 6 (Table 1b) . All six patients developed hypertension,
proteinuria, schistocytosis with elevated LDH. Five out of six had
anemia, thrombocytopenia, elevated creatinine, elevated d-dimer and low
haptoglobin level (Table 1b) . Median six doses (range 4 to 12
doses) of eculizumab were administered to four out of six patients.
Serum eculizumab level was not performed due to unavailability of the
test. Three patients died (2 due to fungal infection and one due to
acute-on-chronic renal failure) (Cases 1 to 3) and all within 3
months upon diagnosis of TA-TMA. Mortality rate was 50%. All three
survivors (Cases 4 to 6) suffered from chronic kidney disease
(CKD) and one (Case 5) required lifelong dialysis(Table 1c) .