The treatment of malignant histiocytosis
Autor: | A Jr Tseng, CN Coleman, RS Cox, TV Colby, RR Turner, SJ Horning, SA Rosenberg |
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Rok vydání: | 1984 |
Předmět: |
Adult
Male Adolescent Dose-Response Relationship Drug Platelet Count Splenic Neoplasms Immunology Liver Neoplasms Leucovorin Cell Biology Hematology Middle Aged Prognosis Biochemistry Methotrexate Doxorubicin Vincristine Antineoplastic Combined Chemotherapy Protocols Splenectomy Humans Prednisone Female Cyclophosphamide Lymphatic Diseases Aged |
Zdroj: | Blood. 64(1) |
ISSN: | 0006-4971 |
Popis: | 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. |
Databáze: | OpenAIRE |
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