Stem cell transplantation for polycythemia vera.

Polycythemia vera (PV) is a rare disease in children. A 9-year-old male was diagnosed following laboratory results acquired because of an acute appendicitis. Regular phlebotomy was performed for over 2 years followed by alpha-interferon treatment. At the age of 12 years, HLA-matched unrelated stem cell transplantation including T-cell depletion was done. The conditioning regimen consisted of busulfan, cyclophosphamide, and ATG. Chimerism was monitored during the whole post-transplant period. A single dose of donor T-lymphocytes was given at month 3. One year after transplantation, chimerism was complete. The patient is in complete remission and shows no signs of transplant-related morbidity at month 78.