Statement of prior presentations: Presented in abstract form at the 33th annual congress of the European Group for Blood and Marrow Transplantation, Lyon, France, March 28, 2007.
High incidence of haemophagocytic syndrome following umbilical cord blood transplantation for adults
Article first published online: 25 AUG 2009
© 2009 Blackwell Publishing Ltd
British Journal of Haematology
Volume 147, Issue 4, pages 543–553, November 2009
How to Cite
Takagi, S., Masuoka, K., Uchida, N., Ishiwata, K., Araoka, H., Tsuji, M., Yamamoto, H., Kato, D., Matsuhashi, Y., Kusumi, E., Ota, Y., Seo, S., Matsumura, T., Matsuno, N., Wake, A., Miyakoshi, S., Makino, S., Ohashi, K., Yoneyama, A. and Taniguchi, S. (2009), High incidence of haemophagocytic syndrome following umbilical cord blood transplantation for adults. British Journal of Haematology, 147: 543–553. doi: 10.1111/j.1365-2141.2009.07863.x
- Issue published online: 27 OCT 2009
- Article first published online: 25 AUG 2009
- Received 28 June 2009; accepted for publication 22 July 2009
- cord blood transplantation;
- reduced-intensity chemotherapy;
- haemophagocytic syndrome;
- engraftment failure
Umbilical cord blood transplantation (CBT) is widely accepted, but one critical issue for adult patients is a low engraftment rate, of which one cause is haemophagocytic syndrome (HPS). We aimed to identify the contribution of HPS to engraftment failure after CBT, following preparative regimens containing fludarabine phosphate, in 119 patients (median age, 55 years; range; 17–69 years) with haematological diseases. Graft-versus-host disease prophylaxis comprised continuous infusion of a calcineurin inhibitor with or without mycophenolate mofetil. Of the 119 patients, 20 developed HPS within a median of 15 d (cumulative incidence; 16·8%) and 17 of them did so before engraftment. Donor-dominant chimaerism was confirmed in 16 of 18 evaluable patients with HPS. Despite aggressive interventions including corticosteroid, ciclosporin, high-dose immunoglobulin and/or etoposide, engraftment failed in 14 of 18 patients. Of these 14 patients, four received second rescue transplantation and all resulted in successful engraftment. Overall survival rates significantly differed between patients with and without HPS (15·0% vs. 35·4%; P < 0·01). Univariate and multivariate analysis identified having fewer infused CD34+ cells as a significant risk factor for the development of HPS (P = 0·01 and 0·006, respectively). We concluded that engraftment failure closely correlated with HPS in our cohort, which negatively impacted overall survival after CBT.