[Trisomy 13-15. Anatomic-pathologic study of a rare forme with ethmocephaly].
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Biomedical subjects
Publications and source records attributed to J Diebold.
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Fifteen cases of primary pulmonary malignant lymphoma (M.L.) were studied using strict criteria to distinguish them from secondary pulmonary manifestations. All were M.L.'s of the lymphoid B type. Diagnosis on the basis of non specific clinical, X-ray and endoscopic findings if often difficult but can be facilitated by the systematic use of immunocytochemical and ultrastructural techniques. Their evolution is in good agreement with the prognostic data given by the Kiel-Lennert histopathological classification. 14 of the patients had M.L. of low grade malignancy; the one case of high grade malignancy was quickly fatal. A few cases appeared as a complication of a preexisting dysimmune disease (Pigeon breeder's disease, Gougerot-Sjogren disease, Liebow's lymphomatoid granulomatosis).
A laparotomy performed for an occlusive syndrome, disclosed the presence of an ileal lymphoma in a renal graft recipient, 17 years after a first renal transplant. Mesenteric lymph node and spleen involvement were associated. The morphology of the large cells, the type of infiltration of the lymph node simulating metastatic carcinoma or malignant histiocytosis were highly suggestive of a new type of high grade malignancy lymphoma recently described which is called 'large cell anaplastic ML'. The immunolabelling study confirmed this diagnosis, demonstrating the following phenotype CD4, CD25 (TAC), CD30 (Ki-1), OKT 9 (transferrin receptor), EMA and HLA DR positivity. The tumour cells were negative for the other T, B and histiomonocytic markers. This case, of a large cell anaplastic Ki-1 positive lymphoma, expressing CD4, in a renal graft recipient shows that high grade ML in such patients are not all of the B cell type.
Forty-five bone marrow (BM) biopsies have been studied in 30 T-cell malignant lymphoma (ML). According to the updated Kiel classification, these ML comprised 12 low grade ML and 18 high grade ML. BM involvement was not significantly more frequent in low grade ML (41.6 per cent) than in high grade (33.3 per cent). This involvement was discovered in 85 per cent of the cases at the time of diagnosis. A correlation was found between BM and other visceral localizations for histological type in all cases. Infiltrates principally showed a nodular pattern in low grade and a diffuse pattern in high grade ML. Hematopoietic hyperplastic reaction was frequent (66.6 per cent) not correlated to involvement. Clinical staging showed extensive spreading. Our patients had an overall median survival of 40 months, worse in high grade ML (median: 19 months) than in low grade ML (41 months) but not statistically different (p = 0.25). Extranodal localizations are a significant criteria for poor prognosis (p = 0.018). Among them, BM involvement appears to be the most significant criteria (Cox model, p = 0.006). Patients with BM localization had a median survival of 9 months contrasting with 40 months in patients without BM localization (p = 0.007). Thus, BM biopsy is useful for the diagnosis of patients with T-ML and essential to establish the prognosis.
Image analysis with a SAMBA 2005 (ALCATEL-TITN, Co) was used to quantify the Ki-67 stained area percentage in 46 T-cell malignant lymphomas (T-ML), classified according to the updated Kiel classification. This parameter demonstrated correlation with the number of Ki-67-positive cellular profiles (r = 0.88, P less than 0.001) and was more reproducible than cell counting. A significant difference was found between low and high grade T-ML (mean values +/- SEM respectively of 10.20 +/- 1.82 per cent and 25.63 +/- 3.15 per cent). The most interesting findings were that: (1) AILD-type T-ML showed an intermediate proliferation rate (15.55 +/- 2.72 per cent) between pleomorphic T-ML with medium and with large cells (respectively 12.53 +/- 3.64 per cent and 22.43 +/- 3.46 per cent), both of which belong to the high grade malignancy group. This finding is in accordance with the poor prognosis of this subtype despite its classification in the low grade malignancy group. (2) Subclassification of the pleomorphic MLs according to the predominance of small, medium or large cells, demonstrated significant differences between these three subtypes. However, the great overlap of values between pleomorphic T-ML with medium and with large cells, seems to indicate that the subclassification of these two subtypes is less valid. (3) A wide range of values with overlap was observed in AILD-type ML, in pleomorphic with medium or large cells and in lymphoblastic T-ML: for these T-ML with variable survival courses, the Ki-67 area percentage, one parameter of proliferative activity, appears worth studying as a prognostic factor.
The authors list the reasons which make it important and useful to classify non-Hodgkin's malignant lymphomas (ML) and discuss the criteria of a convenient classification. They also describe the principles of updated Kiel's classification and the various entities recognized. These entities are divided into type B and type T ML, each type in turn being divided into low malignancy, high malignancy and rare forms.
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To gain insight into the tissue reactions leading to non-infectious loosening 25 autopsy specimens of femurs with well-fixed cemented femoral components of hip prostheses were analysed histomorphologically and morphometrically. The implant duration ranged from one month to 15 years. With the exception of some focal bone-cement contacts bone and cement were separated by a fibrohistiocytic soft tissue membrane. This membrane contained wear particles predominantly of the bone cement mantle and--less abundant--of the polyethylene cups. The amount of wear particles as well as histiocytes and necroses within the membrane statistically significantly increased with advancing time in situ. The cancellous bone adjacent to the soft tissue membrane often revealed a mainly histiocytic infiltration too, accompanied by bone remodeling in this area. The histiocytic inflammatory reaction at the bone cement interface, which is mainly caused by the accumulation of wear particles is considered to be of major importance for the non infectious late loosening of cemented prostheses.
Granulomas consist of an aggregation of lymphocytes and of phagocytes (polynuclear and histiomonocytes) around a tissular lesion. The cells are provided by the blood and/or the lymph. Other cells develop in the focus. Within the granuloma appear other cells which derive from the previous ones, viz immunoblasts, plasmocytes, macrophages, epithelioid and giant cells. These elements cooperate to destroy the invading agent. Mastocytes, fibroblasts and myofibroblasts join in. In the granuloma these latter give rise to scarring.
Sections obtained from lymphoid tissues fixed immediately after biopsy in Bouin's fluid and embedded in paraplast may be used to visualize the immunosecretory cells with classical direct immunofluorescence. A comparative study of the same fragments processed according to the frozen section method followed by acetone fixation or according to Sainte-Marie's method confirms the value and the advantages of the proposed technique. Early fixation of thin fragments seems to be the main point.
Intracytoplasmic tubuloreticular structures were found in the tumour cells of a human lymphosarcoma. Such structures have been described in a wide variety of conditions in man and in animals, as well as in cell cultures of various origin, but rarely in lymphoid tumours. The different hypotheses concerning their nature are discussed.
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The necessity of correct diagnostics of initial lesions of Hodgkin's lymphoma is underlined. The correct assessment may relate of more than 90% of such observation to 90% of noduler sclerosis. The criteria similar to those of WHO are suggested for the differentiation with mixed-cell or lymphoid preponderance.
The case reported is one of lymphadenopathy due to haemophagocytic sinusal histiocytosis which had progressed for more than 10 years before resulting in death at the age of 58 years. In this advanced form, all superficial and deep nodes were involved. In addition to histiocytic hyperplasia, with haemophagocytosis and lipid overload, there was plasmocytic hyperplasia, i.e. B-lymphocytes, with polyclonal hypergamma-globulinaemia and an increase in the levels of many antibodies, in particular against rubella and the Epstein-Barr virus. The aetiopathological significance is discussed, in particular the role of an infection due to the EB virus.
The authors report two new cases of angiofollicular hyperplasia of the mediastinum (Castelman's tumour). The only clinical peculiarity was a rapid increase in volume. The authors then review about 200 published cases. Emphasising the existence of 3 different histological types (plasma cell form, vascular hyaline form and intermediate form) and on the association in certain cases of hematological disorders such as anemia and hypergamma globulinemia. The significance of this lesion is discussed. An inflammatory theory with reactional hyperplasia of the beta-lymphocytes is proposed. It was supported by a direct immunofluorescent study of two cases (first study of this type carried out on such lesions) which showed polyclonal plasma cell hyperplasia predominating on the IgG.