High incidence of haemophagocytic syndrome following umbilical cord blood transplantation for adults.
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.
Takagi
Shinsuke
S
Department of Haematology, Toranomon Hospital, Tokyo, Japan.
Masuoka
Kazuhiro
K
Uchida
Naoyuki
N
Ishiwata
Kazuya
K
Araoka
Hideki
H
Tsuji
Masanori
M
Yamamoto
Hisashi
H
Kato
Daisuke
D
Matsuhashi
Yoshiko
Y
Kusumi
Eiji
E
Ota
Yasunori
Y
Seo
Sachiko
S
Matsumura
Tomoko
T
Matsuno
Naofumi
N
Wake
Atsushi
A
Miyakoshi
Shigesaburo
S
Makino
Shigeyoshi
S
Ohashi
Kenichi
K
Yoneyama
Akiko
A
Taniguchi
Shuichi
S
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
D018952:Antigens, CD34