Biomedical subjects
Publications and source records attributed to G Tricot.
This is a case report of a patient with invasive thymoma. Subsequently to radiotherapy and steroids, a combination chemotherapy of cis-platinum (CDDP) and doxorubicin was tested with good results. The patient later developed a chronic lymphocytic leukemia (CLL); morphologically and cytochemically a T-CLL with skin localizations, hepatomegaly, and a high leukocyte count.
A total of 483 adult patients with non-leukemic non-Hodgkin lymphomas (NHL) were reviewed for neuromeningeal (NM) involvement. NM involvement was found in 20 patients (4,1%). Presenting complaints were mainly headache (9 cases) or diplopia (8 cases). Cranial nerve palsies were frequent (12 cases). CSF examination was abnormal in 76% of the patients and was the most reliable diagnostic procedure, but abnormal cells were found in only 50% of the cases. The pattern of the lymphoid proliferation was diffuse in 90% of the cases. The most frequent histological subtypes of NHL were diffuse histiocytic (10 cases) and diffuse lymphocytic poorly differentiated (6 cases). In 65% of the cases, the lymphoma was initially disseminated. NM involvement was inaugural in 20% of the cases and was the only site of proliferation in 35% of the cases. Median survival after the NM localization was only 3 months. Long remissions were achieved in 3 patients with isolated NM involvement. These results are compared to previously published data. The indications of CNS prophylaxis are discussed.
A 5q- anomaly associated with other chromosome anomalies was found in the infiltrated bone marrow of a patient with a highly malignant teratoma originally located in the mediastinum. There was no evidence of a second malignancy, and it is likely that the 5q- anomaly was, indeed, associated with the malignant teratoma cells.
In a series of 365 consecutive ANLL cases of which 45.1% had abnormal karyotypes, 13 cases were detected with a structural abnormality of the long arm of chromosome 11. Besides one isochromosome 11q, there were six deletions and six translocations. Of these 12 patients, seven had acute monocytic leukemia (FAB-type M5), two had an M4, two had an M2, and one case of secondary leukemia had an M3-like disorder. Similar results with regard to the type of leukemia were obtained upon analysis of 41 cases of ANLL with an 11q anomaly described in the literature. This study confirms that a high proportion of acute monocytic leukemias and a lesser proportion of acute myelomonocytic leukemias are characterized by an 11q anomaly, mostly involving bands q22 and/or q23. Acute monocytic leukemia with an 11q structural anomaly appears to have a poor prognosis.
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Acute hypercalcemia occurred during the following-up of a 70 years old man with Waldenström's Macroglobulinemia (WM). Hematological data were still unchanged in spite of stopping treatment 16 months ago. It is an unusual complication in chronic lymphoproliferative disorders (except, multiple myeloma) and it was never described in the course of WM. It was related to the emergence of diffuse immunoblastic lymphoma, as it has been termed Richter syndrome in chronic lymphocytic leukemias. In WM, this occurrence is less known, but symptoms and prognostic are similar. It is to be pointed out that the spontaneous decrease of the amount of monoclonal IgM is an ominous sign of this immunoblastic transformation. The pathogenesis of hypercalcemia is discussed: the eventuality of a tumoral osteoclast activating factor is suggested.
Anomalies of both No. 3 chromosomes, of the t(3q-; 3q+) type can be observed in human malignancy as reported previously. It is our experience that this anomaly is found predominantly in myeloproliferative disorders, as a rather rare event, though occurring more frequently than similar exchanges between other homologous chromosomes. Previous claims about a relationship between this anomaly and thrombocytosis could not be confirmed, but the features found in a few patients indicate that further research should be undertaken to clarify this point.
The case-reports of 30 patients (16 men and 14 women) with non-Hodgkin lymphoma revealed by a mediastinal mass are reviewed. The tumour was apparently localized (stages I and II) in 63% of the cases, and was associated with pleural effusion in 50%. Two histological types were identified: diffuse lymphoblastic lymphoma and diffuse large cell lymphoma, including 10 cases where the large cells had irregular nucleus and clear cytoplasma and were accompanied by fibrosis. Lymphoblastic lymphomas differed from large cell lymphomas in that they were more common in men and had greater tendency to dissemination, particularly in the meninges and bone marrow. The median survival time was 15 months, but a longer survival could be obtained in patients in complete remission. There was no difference in prognosis between localized and disseminated lymphomas. Radiotherapy of the mediastinum had no effect on survival.
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An adult patient with Sudan Black B (SB B) positive leukaemic lymphoblasts is described. Peroxidase and naphtol AS D chloroacetate esterase stains were negative. The diagnosis of acute lymphoblastic leukaemia (ALL) was based on morphology (FAB classification: L1), on immunological marker studies (cALL+, Tdt+, Ia+) and on electron microscopy, revealed blasts, compatible with lymphoblasts. Additional proof of the diagnosis of ALL were the diffuse lymphadenopathy and the rapid response to ALL chemotherapy. Scattered azurophilic granules were present in some lymphoblasts; ultrastructurally multiple lysosomal inclusions were detected, containing small vesicles, sometimes in association with entrapped cytoplasmic organelles. The large amounts of phospholipids in these inclusions explain the Sudan Black B positivity. This type of ALL can easily be misdiagnosed as acute myeloid leukaemia.
3 patients with preleukaemia (idiopathic acquired myelodysplasia) presenting with isolated peripheral thrombocytopenia and an increased number of megakaryocytes are described. The diagnosis of preleukaemia, however, was strongly suspected on bone marrow morphology, revealing an abnormal megakaryocytic series. Megakaryoblasts with characteristic nuclei and basophilic pseudopodes, and immature megakaryocytes were frequent. These last cells had 1 or 2 nuclei and a basophilic poorly granulated cytoplasm, often containing large vacuoles, corresponding to dilated interconnecting demarcation membranes in electron microscopy. Megakaryocytes were not considered able to produce a normal amount of platelets, explaining the peripheral thrombocytopenia; platelet survival (51Cr) was indeed too long for ITP. Cytogenetic analysis confirmed the diagnosis of an abnormal haematopoietic clone. Ferrokinetics, in vitro agar cultures, bone marrow biopsy and platelet function tests were helpful additional examinations for establishing the correct diagnosis. It is important to differentiate preleukaemia from ITP. Prognosis is totally different and the treatment is not only ineffective in the great majority of the preleukaemic patients, but may be hazardous.
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Richter's syndrome (RS) has been defined as "histiocytic" lymphoma (HL) or Hodgkin's disease (HD) supervening in the course of chronic lymphocytic leukemia (CLL) and related disorders. The clinical, histologic, and immunologic findings in 25 cases (11 women, 14 men) of RS are presented. The initial diagnosis was CLL in 19 cases, diffuse well-differentiated lymphocytic lymphoma in 2 cases, and Waldenstrom's macroglobulinemia in 4 cases. The interval between the initial diagnosis and that of RS ranged from 0 (two cases) to 120 months (median 49 months). At the time of diagnosis of RS, the initial lymphoproliferative disorder was in apparent complete remission in only two cases. The lymphoma was disseminated in at least 18 cases. The overall median survival was four months, but complete remission was achieved in six cases and has been maintained for 15 to 77 months. In four of these six cases, the RS was localized. The histologic diagnosis of HD was made in only two cases. In the other 23 cases, the diagnosis was HL, but in five of these cases, the proliferation was heterogeneous and was considered as an early aspect of HL. Immunologic studies of lymph node cell suspensions were performed in seven cases. In all cases, the B-lymphocytic origin of the lymphoma cells could be ascertained. Detailed studies in four cases showed that lymphoma cells carried SIg of the same isotype and light chain type as that of SIg detected on CLL cells or of monoclonal serum Ig. In these cases, the lymphoma was actually related to the initial B-cell chronic lymphoid disease.
Of the 14 patients with aplastic anaemia treated in our hospital with anti-thymocyte globulin (ATG), four were refractory to random platelets before therapy due to the presence of leucocyte antibodies. In contrast to the ten nonrefractory patients in whom no success was obtained, three of the four refractory patients showed haematological improvement after ATG. Additionally, two patients could be substituted again with random platelets. The other two hardly needed platelet-transfusions after ATG, and they were given HLA-compatible platelets. To determine the degree of immunosuppression, these four patients were tested for the presence of antibodies against leucocytes and two endemic viruses, i.e., mumps and rubella virus. Before ATG, all sera reacted with almost the whole leucocyte testpanel. After treatment, the leucocyte antibodies disappeared completely in three patients. In one patient there was no dramatic change. In all patients, however, the antibody-titre against the mumps and rubella viruses remained constant and there was only a slight decrease in total IgG content in the three "suppressed" patients. We conclude that it might be worthwhile to study systematically the selective immuno-suppressive effect of ATG.
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