The treatment of malignant histiocytosis

A. Tseng, C. N. Coleman, R. S. Cox, T. V. Colby, R. R. Turner, S. J. Horning, S. A. Rosenberg

Research output: Contribution to journalArticle

31 Scopus citations

Abstract

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-one 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 <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.

Original languageEnglish (US)
Pages (from-to)48-53
Number of pages6
JournalBlood
Volume64
Issue number1
DOIs
StatePublished - 1984

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

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    Tseng, A., Coleman, C. N., Cox, R. S., Colby, T. V., Turner, R. R., Horning, S. J., & Rosenberg, S. A. (1984). The treatment of malignant histiocytosis. Blood, 64(1), 48-53. https://doi.org/10.1182/blood.v64.1.48.48