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A man with histologically unequivocal mixed-cellularity Hodgkin's disease, stage IVB, developed an IgG lambda paraproteinemia with bone marrow plasmocytosis but without any other evidence of multiple myeloma. This unusual finding is discussed in the context of recent data suggesting a B-cell origin for some cases of Hodgkin's disease as well as in relation to the evidence of immunoregulatory disturbances in this entity.  相似文献   

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Von Hippel-Lindau (VHL) disease is an autosomal dominant inherited disorder with a predisposition to develop a wide variety of lesions: retinal, cerebellar, spinal and medullar hemangioblastomas, renal cell carcinomas, phaeochromocytomas, and renal, pancreatic and epididymal cysts are the most frequent manifestations of the disease. The prevalence of VHL disease has been estimated to be 1 per 36,000 persons. We report the case of a 68-year-old woman with Von Hipple-Lindau disease who developed high fever with pulmonary and hepatic lesions proven to be Hodgkin's disease on biopsy. To our knowledge, this is the first report of Hodgkin's disease in a patient with Von Hippel-Lindau.  相似文献   

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INTRODUCTION: The association psoriasis and osteomalacia is very rare. EXEGESIS: We report the case of a young girl, aged 17, who was consulting for ostealgia and erythematic and squamous cutaneous lesions. She had a staturo-ponderal delay, ostealgia and a bending of the general state. The osteo-articular examination showed a sternal protrusion with painful limitation of the movements of all joints. The dermatologic examination noted a dry erythrodermis to hick squamous with scalp's crush. The biological (hypocalcaemia, diminished urinary calcium) and radiological (demineralized bone with Looser-Milkmann fractures) results suggest an osteomalacia, diagnostic confirmed by the bone histological study that found out osteoid lines. The cutaneous biopsy was compatible with psoriasis diagnosis. A vitamino-calcic treatment associated to local nursing was undertaken. The evolution was favorable, noted by a cutaneous bleaching with a return back of the articular function. The present receding is of nine years. CONCLUSION: Different studies underlined that outside of its action on the phosphocalcic metabolism, the vitamin D acts on the skin, especially on the proliferation of the keratinocytes that allowed its indication in the treatment of the psoriasis.  相似文献   

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This report presents a case of occult constrictive pericarditis and mitral valve insufficiency following chest radiotherapy. A 44-year-old man had received radiotherapy for the treatment of Hodgkin's disease 8 years ago. At age 40 years, effusive pericarditis occurred and he was treated with intrapericardial drainage. Biopsy revealed a fibrotic and thickened pericardium. He developed congestive heart failure 3 years later. The patient was found to have occult constrictive pericarditis and mitral valve insufficiency. He underwent mitral valve replacement, tricuspid annul plasty, and pericardiectomy. Although there is the benefit of cure for the Hodgkin's disease, the prognosis after treatment is affected by radiotherapy-induced heart disease. After radiotherapy of the chest and mediastinum, long-term cardiological follow-up is recommended in order to detecting patients with radiation-induced heart disease, such as the present case.  相似文献   

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While an association between blood eosinophilia and endomyocardial disease has been recognized, the role of the eosinophil in the pathogenesis of the cardiac lesions remains uncertain. In a 69-year-old-man with large cell carcinoma of the lung, marked eosinophilia was stimulated by and progressed with the course of the neoplasm which was producing an eosinophil chemotactic factor. Peripheral blood eosinophils were vacuolated and degranulated while those in the bone marrow were morphologically normal. Clinical evidence of cardiac dysfunction developed one month prior to death. At autopsy, 12 months after the onset of symptoms, endomyocardial disease was present. There were numerous eosinophils in the damaged myocardium and surrounding the pulmonary neoplasm. In patients with endomyocardial disease and eosinophilia, the eosinophil may be directly cardiotoxic or a primary mediator of cardiac damage; therapeutic attempts to reduce the number of eosinophils might be benefit.  相似文献   

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Cholesteryl ester storage disease (CESD) is a rare disorder of familial incidence characterized by the accumulation of cholesteryl ester and triglycerides in the liver, intestine and bone marrow. Until now only 21 cases have been reported in the literature. We present a 9 months old girl presenting with increased abdominal girth. She had normal liver function tests and increased cholesterol and triglycerides serum levels. The liver biopsy showed many cholesterol cristals seen as needle shaped cristals under polarized light. This is the youngest patient being diagnosed clinically in the literature.  相似文献   

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We report a case of active tuberculosis in a patient with Hodgkin's disease. The two diagnoses were established simultaneously at the Respiratory Disease Unit of the Yalgado National Hospital, Burkina Faso. The clinical presentation was misleading as the signs and symptoms of the two disease are similar. Certain diagnosis was established after isolation of tuberculosis bacilli and histocytology findings for Hodgkin's disease. Both diseases are potentially curable. The clinical course depends on early diagnosis and treatment.  相似文献   

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Most esophageal malignancies are either squamous carcinomas or adenocarcinomas arising in the background of Barrett's esophagus. We describe a case of an 85-yr-old woman in whom the diagnosis of esophageal malignancy was difficult to confirm despite its endoscopic appearance and previous biopsies. This case illustrates the difficulty in diagnosing Hodgkin's disease of the esophagus. Despite the rarity of this entity, if clinically indicated by symptoms, large, deep biopsies by rigid esophagoscopy should be considered.  相似文献   

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The authors report the case of a 21 year old woman admitted to hospital for congestive cardiac failure due to concentric hypertrophic cardiomyopathy. Echocardiography showed severe systolo-diastolic left ventricular dysfunction without obstruction to ejection. Neurological examination showed a stato-kinetic cerebellar syndrome, a posterior radiculo-cordonal syndrome and a dysmorphic syndrome which characterise Friedreich's disease. In the light of this case and a review of the literature, the authors underline the rarity of cardiomyopathy in Friedreich's disease, its particular presentation and its poor prognostic significance in this disease.  相似文献   

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A J Boulton  D N Slater    B W Hancock 《Gut》1982,23(3):247-249
A 57 year old woman developed severe diarrhoea during chemotherapy for Hodgkin's lymphoma. Extensive colonic ulceration was observed at necropsy and electron microscopy showed intranuclear virions consistent with herpesvirus infection. Antibody titres and immunoperoxidase studies indicated that the virus was probably herpes simplex.  相似文献   

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