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Treatment of hemophagocytic lymphohistiocytosis with chemotherapy and bone
marrow transplantation: a single-center study of 22 cases
S Blanche, M Caniglia, D Girault, J Landman, C Griscelli and A Fischer
Departement de Pediatrie, Hopital des Enfants-Malades, Paris, France.
Twenty-two children with hemophagocytic lymphohistiocytosis were treated
with a chemotherapy regimen consisting of VP16-213, corticosteroids, and
intrathecal methotrexate. A sustained clinical and biologic complete
remission was obtained in 15 children and a partial remission in one child;
six children died early of opportunistic infection (n = 4) or of disease
progression (n = 2). Of the 16 children who were placed in first remission,
10 received maintenance chemotherapy alone, while six underwent bone marrow
transplantation (HLA matched in five, HLA mismatched in one). Of the
children who received chemotherapy alone, only two are in long-term
remission after cessation of treatment. The remaining eight patients
relapsed after a mean period of 5.4 months (range 2 to 8 months). Further
treatment using the same regimen induced second remissions of short
duration; death occurred after a median period of 2.3 months (range 0.5 to
6 months). A total of nine patients received allogeneic bone marrow
transplantation (BMT). Among the six children transplanted in remission,
four are in long-term unmaintained remission, 1 to 6 years after
HLA-matched BMT. However, the relapse that occurred in one patient 1 year
post BMT is difficult to interpret because the donor, the patient's
5-year-old sister, also developed the disease 1 year later. An
HLA-nonidentical BMT resulted in unmaintained remission for 1 year, with
autologous hematologic reconstitution followed by disease relapse.
HLA-nonidentical BMT failed in three other patients with active disease at
time of transplant. The poor long-term results of chemotherapy alone
justify the use of related HLA-matched BMT in complete remission.
Volume 78,
Issue 1,
pp. 51-54,
07/01/1991
Copyright © 1991 by The American Society of Hematology
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