[Lymphadenopathy due to sulfasalazine in rheumatoid polyarthritis].
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Biomedical subjects
Publications and source records attributed to N Horschowski.
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The role played by T lymphocytes in myelopoiesis has been established in in vitro studies. Dysregulation of the lymphoid system results in quantitative and, more rarely, qualitative abnormalities of myelopoiesis. The authors report the clinical data of two cases of peripheral T cell lymphoma associated with an AREB type of dysmyelopoiesis. The fact that both conditions were diagnosed simultaneously and progressed in parallel is an indirect argument in favour of the regulation of myelopoiesis by the T lymphocytes.
In this report we rate a new, third-generation automated hematology system (Technicon Instruments H-1) that can furnish a full range of values, including erythrocyte parameters and a leukocyte differential count. Particular attention is focused on erythrocyte morphometric parameters, including measurement of cell size and hemoglobin content on a cell-by-cell basis. We assess the usefulness of new parameters derived from these measurements, such as mean corpuscular volume and red blood cell distribution width, which characterize cell size, and mean corpuscular hemoglobin concentration, and hemoglobin distribution width, which characterize cell hemoglobinization in evaluating normal and abnormal subjects. The value of these parameters in classifying anemias is assessed in our patient population that includes those with iron deficiency anemias and thalassemias, as well as other forms of anemia.
Twenty-eight patients with malignant lymphoma were treated with high dose chemo or chemoradiotherapy and allogeneic or autologous bone marrow transplantation. They can be divided in two groups: Group 1: (19 pts) consisted in patients in relapse or in n complete remission (n greater than 2) (high risk patients); Group 2 (9 pts) consisted in patients in first or second complete remission at time of bone marrow graft (standard risk patients). Complete remission was achieved in 11/17 patients evaluable for response (65%). Duration of response is very different for two groups: in group 1, all patients relapsed within a median of 2 months (range: 1-12) and died within a median of 7 months (range: 2.5-15). In group 2, 7/9 are alive and well in unmaintained CCR in a median of greater than 18 months (range: greater than 15- greater than 36) (P less than 0.01). This experience shows the feasibility of this approach, the obvious antitumoral activity of these conditioning regimens and invited us to use such therapy at an earlier stage of the disease.
Castelman described as angiofollicular hyperplasia (AFH) a benign lymphovascular hyperplasia forming a single tumour, classically situated in the mediastinum. A multifocal lymph node form of AFH was individualised by Leibetseder and Turner about 10 years ago (MAFH). This is a rare syndrome, the clinical and biological characteristics of which are almost identical to angioimmunoblastic lymphadenopathy (AIL). The only difference is in the histology of the ganglia which shows changes of AFH. We report two cases of MAFH. In one patient with histological confirmation of splenic involvement the evolution was subacute. In the second case, the histological features of the lesions were observed to change during successive biopsies: appearances of AFH changed to typical AIL. This observation suggests that MAFH may be a disorder of the immune system. Usually considered as benign lymphatic hyperplasia with a chronic evolution, the long-term development of lymphoma poses the problem of the evolutionary potential of this condition, which may be likened to AIL in which lymphomatous transformation is also recognised.
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29 cases of non Hodgkin's lymphoma (L.N.H.) involving the gastrointestinal (G.I.) tract were reviewed. Primary G.I. involvement was found in 15 patients (2 occurred after Hodgkin's disease), secondary G.I. involvement in 14 patients. Clinical, barium x-ray studies and endoscopic data, prognostically features, mode and results of therapy were analysed in the two groups. Patients were staged using modified (stage IIE) Ann Arbor system; tumors were classified by the "working formulation of N.H.L. for clinical use" (1982). Patients with primary G.I. stage I E - II E N.H.L. were treated with surgery and radiotherapy or surgery with systemic multiple drugs therapy for patients at high risk for recurrence. Disseminated disease and secondary G.I. N.H.L. were treated by chemotherapy; palliative surgery, with high mortality rate, and radiotherapy were occasionally indicated. 3 patients died and 3 had recurrences (5/6 within 2 years) in the first group. Only 2 patients achieved complete remission in secondary G.I.N.H.L. (median survival time: 14 months). Coeliotomy is necessary for unproved diagnosis or emergencies. This study would indicate that the role of surgery remains important in primary localized G.I. stage I E - II E N.H.L.: although diagnosis might be clearly established on endoscopic biopsies; despite results of primary chemotherapy or radiotherapy on controlling local tumor as reported by others authors. Surgical exploration was an essential step in establishing the extent of disease to plan therapy. Resection prevented the possible local complication associated with primary radiotherapy or chemotherapy. The relative risk of treatment induced second malignancies must be considered in the design of adjuvant therapy.
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Modern physiopathological studies of the lymphoid system have enabled accurate immunological identification of the different B lymphopathies. Concurrently, the categorization of T proliferations is evolving. In addition to the morphologically well known categories of T lymphomas, three different histological entities are at present termed T lymphomas by anglo-saxon authors: lymphoma with pleiomorphic cytology, or large cell lymphoma, whose identification seems to be the most difficult; Pinkus lymphoma, with subcutaneous clinical localizations which differ from the initial sites of lymphomas, composed of lymphoid cells whose morphology is very characteristic; Lennert lymphoma, whose precise nature is still under debate, but that has been immunologically identified as a T neoplasm by some American authors. We report four observations that exemplify the complex identification of these lymphomas. One case is a Lennert lymphoma which developed into T immunoblastic lymphoma rising the problem of the relationships between these two diseases. The link between these diverse histological pictures is the T immunological identification of the proliferation for which the routine use of monoclonal antibodies should prove helpful.
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We report three cases of malignant T cell lymphoma of peripheral T lymphocyte origin. This morphological entity was first described by Lennert and Waldron. The diagnosis based on morphological criteria only is difficult because of the many lymphoid malignancies. Immunological characterization is necessary for the identification of the proliferative T lymphocyte subset with a panel of T monoclonal antibodies. Correlations were established between immunological phenotype and clinical and evolutive aspects which are particularly varied in T lymphoproliferative disorders.
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The occurrence of a large cell lymphoma in the course of chronic lymphocytic leukemia is rare and its evolution is fatal at short term. This is the definition of Richter's syndrome. The authors report three cases documented by cytology, histology. The immunological study was performed for one of these by typing on a suspension of fresh bone marrow and lymph node cells. It showed the immunological identity of the chronic phase and the transformation phase (Richter's syndrome). These findings are in line with recent studies showing the immunocellular filiation of the two hemopathies despite the morphological differences, the transformation phase showing the differentiation of the B clone of the chronic phase.
In adults, chronic aquired pure red cell aplasia (CAPRCA) with no thymic tumor may be idiopathic, fitting the description of Kaznelson syndrome, or a preleukemic disorder which will develop into a malignant hemopathy. Diagnosis of the second possibility is difficult to establish at onset. We report a case with a course suggestive of a possible relationship between CAPRCA and acute leukemia.
We report one case of T cell type lymphoma with multilobated nuclei, a distinct variant described by G. Pinkus. The soft palate and subcutaneous tissues were the successive localisations. Despite an aggressive chemotherapy with autologous bone marrow transplantation, a fatal course happened in 18 months. This entity is different from cutaneous lymphomas, mantle zone lymphomas of Waldron, Japanese T lymphomas and from the cases described recently by Weisenburger. The link between these different anatomo-clinical and histological varieties is their belonging to the T cell line.