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.