Biomedical subjects
Publications and source records attributed to E P Gétaz.
A patient with typical chronic lymphocytic leukemia (CLL) who developed central nervous system involvement is presented. The CLL was of B-cell origin and the neurological involvement consisted of perivascular infiltration in the spinal cord.
Two patients with anaplastic carcinoma of the thyroid following irradiation for Hodgkin's disease are presented, and the previously reported cases briefly reviewed. The risk of late development of thyroid carcinoma is life-long and high-dose irradiation in patients with prolonged life expectancy may result in anaplastic carcinoma as well as less malignant histological forms.
Post-irradiation carcinoma of the thyroid is usually histologically well-differentiated. In general, those subjects who developed carcinoma had been exposed to low-to-moderate doses of irradiation for benign conditions. We reviewed the charts of 520 patients with Hodgkin's disease seen at Roswell Park Memorial Institute, and found 2 cases of anaplastic carcinoma amongst other thyroidal abnormalities. The existing reports of post-irradiation carcinoma are reviewed and suggestions are made for the management of heavily irradiated, potentially cured patients with Hodgkin's disease.
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Tuberculosis often complicates lymphoma, and bone marrow necrosis has been described in disseminated tuberculosis. However, the association of lymphoma, disseminated tuberculosis and bone marrow necrosis is rare. We report a patient with this triple association. After a 3-week influenza-like illness the patient was admitted to hospital semicomatose with pancytopenia and hyponatraemia. During routine examination a bone marrow trephine biopsy revealed diffuse lymphomatous infiltration with scattered necrotic foci. On Ziehl-Neelsen staining these foci exhibited numerous acid-fast bacilli. The patient subsequently died and at autopsy was found to have widely disseminated non-reactive tuberculosis.
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The clinical and haematological features of haemophilia are reviewed and the historical aspects, pathogenesis and genetics are discussed. The problems associated with management, such as factor replacement, home therapy, physiotherapy, surgical intervention and the presence of inhibitors, are fully described. Finally, mention is made of the psychological problems of haemophiliacs and of the help given by the South African Haemophilia Foundation.
Historial aspects of the classification of large-cell lymphomas are described. Immunological characterization of the lymphomas has been made possible by identification of T and B lymphocytes according to their cell membrane surface characteristics. The pathogenesis of lymphomas has been clarified by the germinal (follicular) centre cell concepts of Lennert and Lukes and Collins. The various classifications are presented and compared. Whether these subdivisions will have any relevance in the clinical context remains to be seen.
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Twelve cases of non-Hodgkin's lymphoma and a single case of Hodgkin's disease were first diagnosed on bone marrow biopsy. None of the patients had superficially enlarged lymph nodes, and in 3 patients the histological examination of the biopsy specimen showed normal reactive nodes. Eight patients were over the age of 60. The differential diagnosis from benign nodular lymphoid hyperplasia is discussed, with emphasis on the cytology and the paratrabecular position of the lymphoid infiltrate. Ten patients had focal involvement of the bone marrow and 1 of the 3 patients with diffuse involvement had Hodgkin's disease. Lymphoid nodules occur normally in the bone marrow and we conclude that non-Hodgkin's lymphoma and Hodgkins's disease can arise primarily in the bone marrow.
A patient with Waldenström's macroglobulinaemia, whose father died of the same condition, is presented. The literature on familial occurrence of paraproteinaemia is briefly reviewed.
A tenth case of extramedullary plasmacytoma (EMP) of the colon is reported. The term 'primary isolated extra-medullary plasmacytoma' is preferred to those terms previously used. A broad classification of plasmacytoma of the gastro-intestinal tract is suggested and a management policy, based on this classification, is outlined.
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Newer concepts in the application of cytochemistry to the diagnosis of haematological malignancies are discussed. The underlying physiological principles and location of the various enzymes are indicated. Older stains, once thought to be diagnostic, have been shown to be nonspecific. Newer, more accurate stains are now available.
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A patient with Lennert's lymphoma (malignant lymphoma with a high content of epithelioid histiocytes) is described. The case is unique in that hepatosplenomegaly was the presenting feature, the diagnosis was made on bone marrow biopsy and the disorder appears to have run a benign course.
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