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Successful correction of hemophagocytic lymphohistiocytosis with related or
unrelated bone marrow transplantation
KS Baker, CA DeLaat, M Steinbuch, TG Gross, RS Shapiro, B Loechelt, R Harris and AH Filipovich
Section of Pediatric Hematology/Oncology and Bone Marrow Transplant,
University of Nebraska Medical Center, Omaha 68198-2168, USA.
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder of
immune regulation leading to widespread lymphocytic and hemophagocytic
infiltration of vital organs. Apparent cure has only been achieved with
allogeneic bone marrow transplantation (BMT). This report describes 20
consecutive patients, who underwent either matched sibling donor (n = 4) or
unrelated donor (URD; n = 16) BMT. Age at the time of BMT was 0.4 to 5.3
years (median, 0.8 years). Central nervous system disease was present at
diagnosis in 13 patients. At BMT, 14 patients were in a clinical remission,
whereas 6 patients had active HLH. All patients were engrafted after
cytoreduction with busulfan, cyclophosphamide, and etoposide. The
probability of grade II-III acute graft-versus-host disease (GVHD) for all
patients was 57% (95% confidence limit [CL], 0.28, 0.86), and 73% (95% CL,
0.44, 1.0) in URD patients. The overall probability of survival at 3 years
was 45% (95% CL, 0.23, 0.67) and 44% (95% CL, 0.19, 0.68) when URD BMT was
evaluated separately. Favorable BMT outcome was associated with clinical
remission status at the time of BMT. The preparative regimen was well
tolerated, and in the 9 surviving patients it provided durable engraftment
and was effective at eradicating the underlying disease.
Volume 89,
Issue 10,
pp. 3857-3863,
05/15/1997
Copyright © 1997 by The American Society of Hematology
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