title: High incidence of haemophagocytic syndrome following umbilical cord blood transplantation for adults.
abstract: 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.
authors.affiliation: Department of Haematology, Toranomon Hospital, Tokyo, Japan.
authors.forename: Shinsuke
authors.initials: S
authors.lastname: Takagi
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Masuoka
authors.forename: Naoyuki
authors.initials: N
authors.lastname: Uchida
authors.forename: Kazuya
authors.initials: K
authors.lastname: Ishiwata
authors.forename: Hideki
authors.initials: H
authors.lastname: Araoka
authors.forename: Masanori
authors.initials: M
authors.lastname: Tsuji
authors.forename: Hisashi
authors.initials: H
authors.lastname: Yamamoto
authors.forename: Daisuke
authors.initials: D
authors.lastname: Kato
authors.forename: Yoshiko
authors.initials: Y
authors.lastname: Matsuhashi
authors.forename: Eiji
authors.initials: E
authors.lastname: Kusumi
authors.forename: Yasunori
authors.initials: Y
authors.lastname: Ota
authors.forename: Sachiko
authors.initials: S
authors.lastname: Seo
authors.forename: Tomoko
authors.initials: T
authors.lastname: Matsumura
authors.forename: Naofumi
authors.initials: N
authors.lastname: Matsuno
authors.forename: Atsushi
authors.initials: A
authors.lastname: Wake
authors.forename: Shigesaburo
authors.initials: S
authors.lastname: Miyakoshi
authors.forename: Shigeyoshi
authors.initials: S
authors.lastname: Makino
authors.forename: Kenichi
authors.initials: K
authors.lastname: Ohashi
authors.forename: Akiko
authors.initials: A
authors.lastname: Yoneyama
authors.forename: Shuichi
authors.initials: S
authors.lastname: Taniguchi
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D018952:Antigens, CD34 / Q000032:analysis; D036101:Cord Blood Stem Cell Transplantation / Q000009:adverse effects* / Q000379:methods; D005260:Female; D006084:Graft Rejection; D006086:Graft vs Host Disease / Q000517:prevention & control; D019337:Hematologic Neoplasms / Q000628:therapy*; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D012307:Risk Factors; D016019:Survival Analysis; D018183:Transplantation Chimera; D019172:Transplantation Conditioning / Q000379:methods; D055815:Young Adult
chemical_list: D018952:Antigens, CD34