Case 4:
A 19-year-old male with Ewing Sarcoma followed by treatment related MDS
developed lung GVHD on day +451 after a MUD HSCT. GVHD therapy included
sirolimus, prednisone, bortezomib, solumedrol, rituximab, and ECP. He
responded well to bortezomib, allowing wean from prednisone (Table 1).
He subsequently developed pancytopenia requiring bortezomib dose
reduction and eventual discontinuation. The pancytopenia improved after
discontinuation of bortezomib and he was able to remain steroid-free. He
later developed a GVHD flare and was restarted on bortezomib along with
prednisone, while remaining on sirolimus. Bortezomib was again
discontinued due to pancytopenia and development of peripheral
neuropathy. ECP was initiated with demonstrable initial respiratory
response, but he subsequently experienced acute respiratory failure and
succumbed to multi-organ failure.