[Sipple syndrome and pregnancy].
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Biomedical subjects
Publications and source records attributed to M Patey.
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This report describes three cases of acute malignant myelofibrosis characterized by pancytopenia, absence of splenomegaly, bone marrow fibrosis with an immature cell proliferation and rapidly fatal outcome. The authors investigated the origin of blast cells with the use of immunohistochemical markers on paraffin embedded material with anti-factor VIII, BNH9 and anti-lysozyme. They studied the expression of megakaryoblastic, erythroblastic and myeloblastic differentiation in these cells. They demonstrated the heterogeneity of blast cells which are capable of differentiating along the three hematologic cell lines. The morphometric study showed the mutilating or systematized character of myelofibrosis. The increase in reticulin fiber density compared with normal bone marrow was not significantly different from two other types of myelofibrosis. It would be interesting to correlate a quantitative parameter with the course of this disease in order to evaluate the prognosis and the treatment.
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A cytogenetic study was performed in 63 thyroid tumors after a monolayer short-term culture. Clonal chromosomal changes were found in 47% of carcinomas and 31% of adenomas. Chromosome 7 was altered in 40% of cytogenetically abnormal tumors. The modal DNA index measured in 26 tumors was consistent with the chromosomal mode in 88% of cases. A quantitative morphometric analysis of nuclear features differentiated between diploid thyroid adenomas with or without a single translocation, which suggests that they have different biological properties. Clonal chromosomal changes were observed in 78% of carcinomas with an aggressive behavior, but only 28% of those had no risk factors. The two patients who died early had abnormalities of chromosome 7.
We have correlated imprint cytology findings in thyroid tumors to the results of preoperative fine needle aspiration and operative specimen histology. Specificity of imprint cytology proved greater than that of fine needle aspiration cytology and topographic correlations were particularly helpful. Imprint cytology can improve the intraoperative histologic diagnosis. Because abundant cells are available, imprint thyroid cytology is ideal for teaching and training cytologists. Imprint cytology provides enough cells to perform special techniques, such as quantitative cytology, that are useful for the diagnosis of some tumor varieties (e.g. follicular tumors).
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A case of malignant histiocytosis was studied by cytology, cytochemistry, electron microscopy, and cytogenetics. It was shown that the malignant cells expressed a fully differentiated histiocytic pattern with high macrophagic activity. This correlated with the presence of polyploid metaphases. The significance of polyploid cells in the definition of malignant histiocytosis is discussed.
A 58-year-old man showed bone marrow crystalline structures associated with a lambda light chain producing multiple myeloma. Analysis and processing of electron images clearly displayed the periodic structure of the crystals. Immunochemistry suggested that they contained the whole or a fragmented constant portion of immunoglobulin.
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The case of a 36 year old man who presented prominent bilateral cervical lymphadenopathies is reported. The diagnosis of sinus histiocytosis with massive lymphadenopathy (Rosai and Dorfman, 1969) was retained on account of a massive hyperplasia of foamy histiocytes with PAS + inclusions, lipidic storage and hemocytophagy in the sinuses and cords of the lymph nodes. The patient died after 34 months evolution. At the autopsy an important proliferation by large atypical sometimes multinucleated cells in the lymph nodes, liver, bone and spleen was found, as observed in malignant histiocytosis. The ultrastructural study confirmed the histiocytic nature of the cells. The interest of immunohistochemical studies on paraffin embedded material with characterization of tumor histiocytes with lysozyme and alpha 1 antichymotrypsin is discussed. This report described an unusual form of malignant histiocytosis simulating on his early phase sinus histiocytosis with massive lymphadenopathy.
In Hashimoto's thyroiditis, there is a diffuse interfollicular infiltration of lymphocytes and plasmocytes and lymphoid collections with germinal centers. Eleven cases of Hashimoto's disease were studied with immunocytochemical methods for characterization of intracytoplasmic immunoglobulins (IgA, IgG, IgM, Kappa and lambda light chains). Most of the cells are plasmocytes stained positively for intracytoplasmic IgG; IgA positive cells were less frequent and IgM positive cells are rare. Kappa positive plasma cells are more numerous than lambda positive cells in the germinal centers and the interfollicular infiltration. By an indirect immunofluorescence technique in frozen tissue, we have studied with four monoclonal antibodies (OKT3, OKT4, OKT8, B1) the T cells subsets populations in two cases.
The authors have studied, about a total of 138 exams, the value of parathyroid nuclide scan. All the patients, who had primary hyper parathyroidism, have been operated upon. The results of parathyroid scintigraphy have been compared to the results of surgical exploration. Overall sensibility of the exam has been 80%, better with the MIBI (89%) than with the Thallium (78%). The specificity has also been better with the MIBI (95%) than with the Thallium (87%) with a global result of 88%). Computerized treatment of images with automatized subtraction especially studied in Institut Jean-Godinot, Rheims, explain the quality of these results.
Ten years after nephrectomy a renal cell carcinoma of the felt kidney, the patient presented successively pancreatic, adrenal gland, thyroid gland and contralateral renal metastases. Surgical exeresis was performed for the different metastases with a survival period of 7 years. No similar observation (non-necropsic discovery, surgical treatment, prolonged survival) of multiple metastasis of renal cell carcinoma to endocrine glands was found in the literature. The important role of modern imaging techniques are emphasized together with the need for long-term follow-up of operated renal cell cancer since the clinical course can be unpredictable. Long-term remission has been reported and surgical exeresis of metastasis should always be proposed when possible.
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