[Manifestations of Hodgkin's disease caused by retinoids?].
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Biomedical subjects
Publications and source records attributed to B Desablens.
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A case of sternal plasmocytoma discovered 28 months after an occupational trauma is reported. At the first examination a small quantity of monoclonal IgG was present, and persisted in spite of a rather heavy treatment by irradiation and chemotherapy. To-day, more than eight years after the trauma, there is not any clinical or radiological sign of generalization, but bone marrow plasmocytosis is notably increased. Thus the long-dated prognosis is reserved. We discuss the frequency of solitary plasmocytomas of the sternum, the possibility of generalization sometimes after a very long delay, the relationship between the trauma and the plasmocytoma, the lack of pejorative significance of associated monoclonal dysglobulinemia. We suppose that in our case the dysglobulinemia antedated the trauma, this hypothesis is corresponding to the present concepts on oncogenesis, the trauma being only a cocarcinogenic agent.
A new case of polycythaemia associated with an uterine fibroma is presented. The presence of erythropoietin in the tumor has been demonstrated by an in vitro technique of titration using a microculture of foetal mouse liver cells. The study of 46 previously reported cases show that the usual pattern is that of an isolated polycythaemia occurring in a woman in her fifty and of a large fibroma. The surgical cure of the tumor relieves rapidly and definitively the polycythaemia. The hypothesis of an inappropriate secretion of erythropoietin seems admitted, three factors playing probably a role in the occurrence of the polycythaemia: size of the fibroma, state of the iron stores and presence of an inhibitory factor against erythropoiesis.
Adult Still's disease is a newly described disorder that is of interest not only for the rheumatologist but also for the internist since fever or extra-articular symptoms can reveal this disorder, which appears to be identical to the Wissler-Fanconi's syndrome. We report a new case characterized by the occurrence of a constrictive pericarditis with clinical features of protracted fever, by the late appearance of a Sjögren's syndrome and by peculiar blood abnormalities: hyperleucocytosis up to 80 000 after splenectomy, transient Felty's syndrome, blood monocytosis and bone marrow mastocytosis.
The study of 36 published cases of adult Still's disease shows the following features: the onset takes place usually during the third decade of life, most often as a polyarthralgia characterized by sometimes starting fever, evanescent rash, neutrophil leukocytosis, negativity of the serological tests for rheumatoid arthritis and systemic lupus; lymphadenopathy and splenomegaly are frequent but not constant; serous cavities, particularly pericardium, are rather frequently involved; evolution is characterized by a succession of relapses and remissions, the far prognosis being rather good (1/3 of cures, 1/3 of mild articular relapses, 1/3 of articular sequelae, involving chiefly neck and hips); corticosteroids and heavy doses of aspirin seem to give the best therapeutic results, the usefulness of a maintenance treatment is debatable.
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In this article clinical and neuroradiological features from a cohort of 48 immunocompetent patients who have a histologically proved primary cerebral lymphoma are considered. Our series consisted of 27 men and 21 women with an average age of 59. The clinical results gave 73% patients with a focal deficit, 46% with deterioration of vigilance, 35% intracranial hypertension, and only 8% with epilepsy, which was never revealing. We observed 4 uveitis of which 3 revealed, and preceded by several months, the neuroradiological manifestations. Histological classification using the criterias of the Working Formulation showed that 92% of our patients had a large cell lymphoma (class G or H). Precise analysis of computed tomography features of 40 patients revealed 46 lesions (most of them were isodense) before contrast medium administration. In all the cases, the lesion enhancement was intense and homogeneous. In 50% of the cases, there were multiple lesions. Sixty lesions were of the supra tentorial compartment, lobar in 32 cases, deep in 28 cases. Fifteen were infratentorial. From our experience, we can put forward the following suggestions: 1) Neuroradiological aspects suggesting primary cerebral lymphomas exist but none of them are specific. 2) Research of an uveitis is important, as this makes the histological diagnosis more simple. 3)The prescription of corticosteroids should be delayed until the histological diagnosis is certain because the primary cerebral lymphoma, which is very corticosensitive, is likely to disappear with this treatment and then change the biopsy results.
Intravenous immunoglobulin (IVIG) is currently used for an increasing number of indications where an immune-medicated disorder is suspected. It is considered as a safe and efficacious treatment but several cases of severe acute renal failure (ARF) have been described since 1987. We report four cases of IVIG-induced ARF and the literature on the subject is reviewed. The chronological and semiological characteristics of this rare adverse effect are analysed. A sudden and marked increase of serum creatinine within the 2 to 4 days following institution of IVIG therapy, especially when the patient becomes oligo-anuric, is very suggestive of IVIG renal toxicity. The recovery of renal function is often obtained in 10 to 15 days after discontinuation of the drug. Histological changes are characterized by osmotic nephrosis injuries. Patients generally presented numerous risk factors such as over 65 years, particularly in men, pre-existing renal disease, long-standing diabetes mellitus or hypertension, volume depletion, quick infusion rate, body-weight adjustment of IVIG doses in fat subjects. The mechanism of renal injury remains speculative but an oncotic overloading of kidney probably occurs. These results indicate the need for research and investigation of risk factors before starting IVIG therapy. Close monitoring of serum creatinine and diuresis should be carried out during and after treatment.