Case 2:
A 4-year-old male patient with X-linked lymphoproliferative disorder and
associated EBV-positive DLBCL presented with aGVHD of the skin on day
+78 after haploidentical maternal HSCT. GVHD prophylaxis was with
sirolimus. Steroids were initiated once GVHD developed. Unable to wean
off steroids, bortezomib was initiated (Table 1), during which
prednisone was weaned and eventually discontinued. Subsequently, all
other immune suppression was discontinued. Later, he subsequently
developed intervening liver GVHD at day +341 and restarted bortezomib,
requiring a 50% dose reduction due to neutropenia. Upon neutrophil
count recovery, the bortezomib dose was increased but, liver function
did not improve. After five doses bortezomib was discontinued and
pentostatin initiated. The patient was then successfully weaned from
prednisone. Bortezomib showed promise in treatment of skin GVHD but had
no effect on his liver GVHD.