Faculty Collaboration Database

Unrelated donor hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis. Bone Marrow Transplant 2008 Aug;42(3):175-80

Date

05/06/2008

Pubmed ID

18454181

DOI

10.1038/bmt.2008.133

Scopus ID

2-s2.0-50049118882 (requires institutional sign-in at Scopus site)   91 Citations

Abstract

We report outcomes after unrelated donor hematopoietic cell transplantation (HCT) for 91 patients with hemophagocytic lymphohistiocytosis (HLH) transplanted in the US in 1989-2005. Fifty-one percent were <1 year at HCT and 29% had Lansky performance scores<90%. Most (80%) were conditioned with BU, CY, and etoposide (VP16) with or without anti-thymocyte globulin. Bone marrow was the predominant graft source. Neutrophil recovery was 91% at day-42. The probabilities of grades 2-4 acute GVHD at day-100 and chronic GVHD at 5 years were 41 and 23%, respectively. The overall mortality rate was higher in patients who did not receive BU/CY/VP16-conditioning regimen (RR 1.95, P=0.035). The 5-year probability of overall survival was 53% in patients who received BU/CY/VP16 compared to 24% in those who received other regimens. In the subset of patients with known disease-specific characteristics, only one of five patients with active disease at HCT is alive. For those in clinical remission at HCT (n=46), the 5-year probability of overall survival was 49%. Early mortality rates after HCT were high, 35% at day-100. These data demonstrate that a BU/CY/VP16-conditioning regimen provides cure in approximately 50% of patients and future studies should explore strategies to lower early mortality.

Author List

Baker KS, Filipovich AH, Gross TG, Grossman WJ, Hale GA, Hayashi RJ, Kamani NR, Kurian S, Kapoor N, Ringdén O, Eapen M

Author

Mary Eapen MBBS, DCh, MRCPI, MS Professor in the Medicine department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Female
Follow-Up Studies
Graft vs Host Disease
Hematopoietic Stem Cell Transplantation
Humans
Infant
Lymphohistiocytosis, Hemophagocytic
Male
Probability
Retrospective Studies
Survival Rate
Survivors
Time Factors
Tissue Donors
Transplantation Conditioning