Hematopoietic stem cell transplantation (HSCT) for Langerhans cell histiocytosis (LCH) in Japan

There exists limited information about the usefulness of hemopoietic stem c ell transplantation (HSCT) for the treatment of patients with refractory La ngerhans cell histiocytosis (LCH), We report here four Japanese pediatric p atients with multisystem LCH disease who underwent HSCT between 1994 and 19 97, Two of the four patients are doing well without any relapse, However, n either of them shows improved sequelae 3 to 4 years after allogeneic HSCT, although the graft was rejected in one of the cases, The remaining two pati ents died of septic shock. A review of the literature of II patients reveal ed four fatalities after the use of HSCT in the treatment of LCH, Three of these were due to active LCH and three deaths occurred within 2 months afte r HSCT, To establish the usefulness of HSCT for refractory LCH, further stu dies are required.