THE TREATMENT OF MALIGNANT HISTIOCYTOSIS

  • 1 January 1984
    • journal article
    • research article
    • Vol. 64  (1) , 48-53
Abstract
Twenty-four consecutive cases of malignant histiocytosis (MH) treated between 1973 and 1983 were reviewed. Most patients presented with systemic symptoms (91%) and advanced disease (stage IV, 80%). Multiple organ involvement was common. In 6 cases, pathologic tissue was characterized by frozen section immune histochemistry. One case expressed a mature monocyte/macrophage phenotype; 3 cases were considered null cell or primitive lesions; and 2 cases were identified as probable T cell lymphomas. Seven patients underwent splenectomy. Two patients died prior to 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+ mo.; median duration of PR was 2.4 mo. Median survival for patients attaining a CR has not been reached vs. 3 mo. for the PR and NR groups. For all 24 patients, median survival was 2 yr, with a 5-yr actuarial survival of 40%. Multivariate analysis revealed that a platelet count < 150,000 and the dose of drug delivered 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.