Neurologic abnormalities
He remained asymptomatic but over 9 days, he experienced several
episodes of headache, blurred vision and minor mental status changes,
with fever high up to 38.5. Moreover, Peripheral smear showed an
increased number of schistocytes (1.3%) (Fig. 1).
PEX through a right femoral venous hemodialysis catheter was carried out
daily immediately after the onset of neurologic abnormalities
immediately even if ADAMTS-13 levels remained unknown given the high
risk of morbidity and mortality of TTP within the first 24 h if plasma
replacement therapy is not given 4 However, because
the shortage of serum, we collected 1000ml serum, then added 500ml
volume of albumin. The PEX procedure resulted in a dramatic response
with improvements. His neurologic abnormalities resolved immediately and
did not recur.
On the 3th day post PEX therapy, the PLT rise to 156×109/L with the LDH
level down to 478 U/L. Because complete response of PEX was defined by a
full resolution of any neurological manifestations and platelet count
recovery (>150×109/L) for at least two days based on
previous studies and in accordance with international guidelines5. Therefore, we continued PEX therapy. On the 4th day
post PEX, continuous improvement was noted on the blood test, with
platelets peaked to 195×109/L and LDH down to 331 U/L. However, the
patient presented with a sudden onset right leg swelling and pain. There
were no associated signs or symptoms such as dyspnea or fever. The color
Doppler ultrasound demonstrated evidence of DVT in the right lower leg
which showed total thrombosis of the right external iliac and femoral
veins and nearly total thrombosis of the right popliteal vein (Fig. 2).
We consulted vascular surgeons. Based on their recommendation, the
patient underwent inferior vena cava filter placement and catheter
thrombolysis and perfusion catheter insertion with continued
administration of thrombolytic agent (Fig. 3). At the meantime, PEX
session was sustained for another 2 days. The DVT improved markedly, and
we shifted to an oral anticoagulant (rivaroxaban). After another twice
PEX, he continued to remain asymptomatic, his hematological parameters
stabilized with a platelet count of 200 × 10^9/L at discharge and
plasma D-dimer levels returned to normal. The patient is now under
follow-up in the outpatient clinic and is undertaking rivaroxaban daily,
while progressively tapering oral corticosteroids. In a yearly
follow-up, there has been no anemia and the platelet count also remains
normal to date.