ABSTRACT
Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease (RDD), is a rare clinicopathologic entity of unknown etiology. The majority of patients do not require treatment, demonstrating spontaneous resolution or stable disease. However, in some cases the disorder runs a progressive course and/or threatens vital structures and functions, which dictates some form of intervention. It is not clear what constitutes the best approach for those cases. We report on a girl with an extensive and progressive form of the disease who was cured with combination chemotherapy, showing no evidence of recurrence after a follow-up of 5.5 years.
Subject(s)
Anti-Inflammatory Agents/administration & dosage , Antimetabolites/administration & dosage , Drug Therapy, Combination , Histiocytosis, Sinus/drug therapy , Adolescent , Female , Histiocytosis, Sinus/complications , Histiocytosis, Sinus/pathology , Humans , Lymph Nodes/pathology , Mercaptopurine/administration & dosage , Methotrexate/administration & dosage , Prednisone/administration & dosage , Treatment Outcome , Vinblastine/administration & dosageABSTRACT
The authors describe the disease of a 22-year-old woman treated from the age of 13 years on account of Hodgkin's lymphoma by irradiation and cytostatic treatment. On account of a relapse of lymphoma at the age of 14 years megachemotherapy with subsequent transplantation of autologous bone marrow. In the course of eight years of the follow up gradual development of constrictive pericarditis with exsudate. Concurrently progression of mitral insufficiency based on valvular prolapse resulting from radiation. During the last two years refractory systemic hypertension resistant to treatment. At the peak of the disease development of cardiac tamponade and cardiac cachexia with anasarca. After anamnestic, clinical and haemodynamic analysis total pericardetomy was indicated and mitral valve replacement performed. The operation led to improvement of the patient's condition, systemic hypertension receded completely. The patient is in permanent remission.
Subject(s)
Antineoplastic Agents/adverse effects , Bone Marrow Transplantation , Heart/drug effects , Heart/radiation effects , Hodgkin Disease/therapy , Radiation Injuries , Adult , Combined Modality Therapy , Female , Humans , Mitral Valve Prolapse/etiology , Mitral Valve Prolapse/surgery , Pericarditis, Constrictive/chemically induced , Pericarditis, Constrictive/etiology , Pericarditis, Constrictive/surgery , Radiotherapy/adverse effectsABSTRACT
We describe three cases of sclerosing polycystic adenosis (SPA) of the parotid gland, a salivary condition analogous to fibrocystic disease of the breast. For the first time, immunoreactivity for oestrogen and progesterone receptors was demonstrated, suggesting a possible participation of hormone stimulation in its pathogenesis. In addition, all our cases showed foci of dysplasia of the ductal epithelium, which in one case was severe enough to amount to carcinoma in situ. This feature that has not previously been reported in SPA.
