Pathogenesis of the generalized Shwartzman reaction. Evaluation of platelets as a mediator of the suppressive effect of a nitrogen mustard.
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Biomedical subjects
Publications and source records attributed to R D Collins.
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Major advances have been made in the diagnosis and therapy of childhood leukemia over the last two decades (Zuelzer and Flatz, 1960; Miller, 1980; Steinhorn and Myers, 1981; Kobrinsky et al., 1980). Sophisticated phenotypic analysis of these leukemias has partially revealed their complexity; there are at least three to five distinct clinical pathological entities in the childhood leukemia groups. Improved therapy has resulted in marked increases in survival and cures. Less attention has been given to the pathology of childhood leukemia, although the frequency and complexity of tissue examinations have greatly increased. In particular, the pathologic manifestation of relapses, detection of focal leukemia after treatment and the pathology of complications have not been systematically reviewed. Our goal was to look at all of the pathologic manifestations of leukemia and to relate these manifestations to homogeneous patient populations grouped by their type of leukemia. Therefore all of the cytologic, hematologic, histopathologic, ultrastructural and immunologic materials on a consecutive group of leukemic children seen at Vanderbilt from 1970 to 1981 were reviewed; pathologic manifestations of leukemia were then analysed in relationship to diagnostic categories.
To extend the clinical-pathologic description of small cleaved cell lymphoma (SCCL), we reviewed the records of 106 patients with SCCL who were treated in accordance with a policy of watchful waiting and palliative therapy. Median age was 58 years. A pure diffuse pattern was seen in only 16 per cent of cases. Stage III or IV disease was present in 85 per cent of patients; marrow involvement was noted in 60 per cent of patients. 'B' symptoms were present in 25 per cent of patients. By univariate analysis, age greater than 60, diffuse pattern, stage IV disease, involvement of liver, lung, pleura, or g-i tract, 'B' symptoms, surface heavy chain IgM or IgM-D, and LDH greater than 200 IU/dl were found to be significantly associated with a poor prognosis. Marrow involvement, increased (5-25 per cent) transformed (non-cleaved) cells, and surface light chain had no prognostic significance. By multivariate analysis, only age greater than 60, stage IV disease, and LDH greater than 200 IU/dl were prognostically significant. For patients with all three unfavourable prognostic features, median survival was only 11 months. For patients with age less than or equal to 60, stage I, II, or III disease, and LDH less than or equal to 200 IU/dl, median survival was not reached at 180 months. Recognition of prognostic features in indolent lymphoma can help clarify whether the results of treatment protocols represent true advances or the mere selection of favourable patients.
Some patients with cerebriform T-cell lymphoma (CTCL) undergo morphologic transformation to a large cell lymphoma. From a series of 113 patients with CTCL, 22 patients were identified with transformed CTCL. Stages of involvement at diagnosis were: I (seven), II (four), III (four), IV (seven). Nine patients had transformation at the initial diagnosis while the median time from diagnosis to transformation in the other 13 patients was 16 months (range: 3 months-6 years). Thirteen had transformation extracutaneously: lymph nodes (eight), central nervous system (two), and other extranodal sites (three). T cell markers were identified in all cases; of 15 cases with complete phenotypes, there were eight T-helper, three T-suppressor, and four aberrant T phenotypes. Serology for human T-leukemia virus-I (HTLV-I) was negative in eight patients tested. Median survival from diagnosis was 27 months compared to 53 months in 53 patients without transformation (p = 0.003). Despite combination chemotherapy in 12 patients following transformation, median survival after transformation was 12 months and only 7 months with extracutaneous disease. The likelihood of transformation could not be predicted by the initial histology, immunophenotype, or stage of disease.
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The Mononuclear Phagocyte System, including promonocytes, monocytes, and tissue histiocytes (macrophages), gives rise to moncytic leukaemias and histiocytoses. A classification of these neoplasms by their presumed cell of origin is proposed, and criteria for their diagnosis are given. Histopathological, cytochemical, immunological and ultrastructural features useful in distinguishing these disorders form other haematopoietic or lymphoid neoplasms are presented in detail. The resulting classification reflects our knowledge at this time; as techniques become available to define further functional subpopulations of mononuclear phagocytes, it is likely that this classification will become as diverse as that of lymphoid neoplasms.