[Hepatic lesions in leukosis. Histopathological study].
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Biomedical subjects
Publications and source records attributed to J Diebold.
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Peripheral lymphoid organs have two groups of non lymphoid cells: that of macrophages which belongs to R. van Furth's system of mononuclear phagocytes and the one made of elements for long labelled "reticular cells". The use of electron microscopy has permitted a good understanding of these elements and a recognition of ubiquitous cells (fibroblasts, endothelial cells) and specific cells to the lymph node which seem to play a very important role in the immune reactions (interdigitated cells, reticulum cells, dendritic cells). We describe their light and electron microscopical features and histoenzymatic characters, and present the actual concept of their origin and function. The latest discoveries on their classification are reviewed and have permitted to distinguish three categories of non lymphoid cells: first the group of reticulum cells (reticulum cells and dendritic cells), second that of mononuclear phagocytes (macrophages and interdigitated cells) and third that of endothelial cells. Other elements of questionable existence or insufficiently known do not appear in this classification: dark cells, Steinman and Cohn's dendritic cells and cells containing Birbeck's granules.
In this second part, the dysimmunitary conditions which associate a polyclonal hypergammapathy and adenopathy are studied. The latter simulate a malignant lymphoma both clinically and histologically, hence the term sometimes used: "pseudo-lymphoma". Above all, these dysimmunitary states should be considered as real pretumoral states, and a malignant lymphoma or Kaposi sarcoma can arise at any time during the evolution of the illness. Finally, all these illnesses, even apart from the appearance of malignant tumors, have a severe evolution and death can occur due to consequences of immune disorders and particularly to infection. The different histopathological and evolutive aspects of the following are successively studied: angio-immunoblastic lymphadenopathy; the association of adenopathy with Kaposi's sarcoma; the syndrome of epidemic immune deficiency of T cells (acquired immune deficiency syndrome; frequent in homosexuals; the Gougerot-Sjögren syndrome; adenopathy due to medicaments; and the lymphomatoid granulomatosis of Liebow. The similarity of the histopathological lymph node lesions observed in these different dysimmunitary states suggests an identical physiopathology. Also, the existence of frequent associations between these different diseases suggests a common triggering mechanism. The role of virus on a favourable hereditary ground is discussed.
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The authors discuss the W.H.O. classification of neoplastic disorders of lymphatic and haematopoietic tissue proposed by G. Mathé and H. Rappaport. They note the points in common with previously published new classifications and make a number of remarks and criticisms. They stress the point most open to discussion: maintenance of the concept of reticulosarcoma. They propose the parallel use of several classifications, including the old nomenclature of Rappaport, in routine histopathology during the comming years.
We report an uncommon case of Waldenström's macroglobulinemia with prominent splenomegaly associated with acquired C1 inhibitor deficiency, lupus anticoagulant and red blood cell autosensitization. Each of these immune abnormalities has been occasionally reported separately in patients with diverse B-cell lymphoid malignancies, but never before have they appeared simultaneously in the same patient. In this case, the pathogeny of the C1 inhibitor deficiency is questionable and may possibly be directly related to the IgM monoclonal gammapathy through protein-protein interactions, as occurs for lupus anticoagulant.
We report on a rare case of fatal congenital alveolar capillary dysplasia. The newborn boy of a 37 weeks' normal gestation suffered from persistent pulmonary hypertension without any cardiovascular malformation and died at the age of 4 weeks despite intensive treatment. The autopsy tissue was examined histologically, immunohistochemically, and ultrastructurally. Moreover, a three-dimensional tissue reconstruction based on serial sections was performed comparing the affected lung with normal lung tissue. We observed a unique pattern of pulmonary dysplasia: An extreme decrease of capillaries was localized centrally within thickened intra-acinar septa instead of capillaries intensely neighboring pneumocytes; ectatic veins normally running in the interlobular septa were found to accompany intralobular bronchovascular bundles, denying a clear distinction between pulmonary and bronchial veins; small muscular pulmonary arteries extended to the precapillary level and type 2 pneumocytes exceeded by far the type 1 pneumocytes, inverting the normal ratio. In summary, alveolar capillary dysplasia is assumed to be a primary capillary disorder of unknown origin, which possibly involves the regular differentiation of pneumocytes, according to the close alveolocapillary relationship during pulmonary ontogenesis. We consider the venous alterations as being part of the dysplasia, whereas the arterial phenomena might occur secondarily. Recent reports on affected siblings suggest a genetic component of pathogenesis.
The authors present 3 cases of undifferentiared naso-pharyngeal carcinoma of the Schmincke-Regaud type. They study the anatomopathological, appearances, the geographic distribution, the background, the clinical expression and course, the immunological, virological and genetic factors of this very curious type of cancer the diagnosis and treatment of which remain difficult.