The treatment of malignant histiocytosis
A Tseng , CN Coleman, RS Cox, TV Colby, RR Turner, SJ Horning and SA Rosenberg
Twenty-four consecutive cases of malignant histiocytosis (MH) treated at
Stanford Medical Center between 1973 and 1983 have been reviewed. Most
patients presented with systemic symptoms (91%) and advanced disease (stage
IV, 80%). Multiple organ involvement was common. In six cases, pathologic
tissue was further characterized by frozen section immune histochemistry,
using a panel of monoclonal antibodies known to react with monocytes and
macrophages, as well as a variety of hematopoietic cells. One case
expressed a mature monocyte/macrophage phenotype; three cases were
considered null cell or primitive lesions; and two cases were identified as
probable T cell lymphomas. Seven patients underwent splenectomy. Two
patients died prior to any treatment. Twenty-two patients were treated with
CHOP (cyclophosphamide, Adriamycin, vincristine, prednisone) +/- bleomycin
(B), +/- midcycle high-dose methotrexate (HD-MTX) with leucovorin rescue.
Seven patients received prophylactic intrathecal MTX. Of 22 evaluable
patients, there was a 68% complete response rate (CR), a 23% partial
response rate (PR), and a 9% no response rate (NR). Median duration of CR
was 30+ months; median duration of PR was 2.4 months. Median survival for
patients attaining a CR has not been reached v 3 months for the PR and NR
groups. For all 24 patients, median survival was 2 years, with a 5-year
actuarial survival of 40%. Multivariate analysis revealed that a platelet
count less than 150,000 (P Cox = .005) and the dose of drug delivered (P
Cox = .057) were the most important prognostic factors. Prophylactic
intrathecal MTX therapy and splenectomy did not influence survival.
Although MH is an aggressive disease with a poor prognosis, it is
potentially curable. Systematic and aggressive treatment should further
improve the outcome.
Volume 64,
Issue 1,
pp. 48-53,
07/01/1984
Copyright © 1984 by The American Society of Hematology
