A. Gurgey et al., TREATMENT OF X-LINKED LYMPHOPROLIFERATIVE DISEASE (DUNCAN DISEASE) WITH HIGH-DOSE METHYLPREDNISOLONE AND ETOPOSIDE (VP-16), Turkish Journal of Pediatrics, 38(2), 1996, pp. 217-222
A five-year-old boy in the acute phase of X-linked lymphoproliferative
(XLP) syndrome (Duncan disease) with high fever and hepatosplenomegal
y was treated successfully with high-dose methylprednisolone and VP-16
for 15 months. He had been alive for four years after diagnosis as of
this writing. We recommend high-dose methylprednisolone and VP-16 in
patients with XLP who have to wait for a suitable donor before bone ma
rrow transplantation.