Search

Primary extramedullary plasmacytoma of the skin is a very rare disease; to our knowledge, only a few cases have been published in the world literature. In this paper, a patient who had a primary cutaneous plasmacytoma of the lower lip for 20 years is described. Serological, radiological and histological examinations excluded the possibility of an underlying multiple myeloma. The position of cutaneous plasmacytomas within the group of malignant plasma cell neoplasias is discussed and the relevant literature is reviewed.

A 42-year-old woman had a 10-year history of external ophthalmoplegia, malabsorption resulting in chronic malnutrition, muscle atrophy and polyneuropathy. Computer tomography revealed hypodensity of her cerebral white matter. A metabolic disturbance consisted of lactic acidosis after moderate glucose loads with increased excretion of hydroxybutyric and fumaric acids. Post-mortem studies revealed gastrointestinal scleroderma as the morphological manifestation of her malabsorption syndrome, ocular and skeletal myopathy with ragged red fibers, peripheral neuropathy, vascular abnormalities of meningeal and peripheral nerve vessels. Biochemical examination of the liver and muscle tissues revealed a partial defect of cytochrome-c-oxidase (complex IV of the respiratory chain). This mitochondrial multisystem disorder may represent a separate entity to be classified between the spectrum of myoencephalopathies and oculo-gastrointestinal muscular dystrophy.

The difficulty in differentiating aggressive fibromatosis from well-differentiated fibrosarcoma is described, and is illustrated by a case report. Therapeutic possibilities are also discussed.

Sodium taurocholate pancreatitis in the rat is a frequently used experimental model for evaluating therapeutical regimes in this disease. It is, however, uncertain when treatment should be started, as the early phase of this experimental model and thus the time when the pancreatitis really develops is unknown. Serum and pancreatic enzymes, as well as pancreatic morphology, were therefore studied 5, 30, and 60 min after induction of sodium taurocholate pancreatitis. It was found that increase in serum enzymes and decrease in pancreatic enzymes and morphological changes characteristic for acute pancreatitis develop as early as 5 and 30 min after induction of pancreatitis. Thus, therapy in this model may be started shortly after induction of acute pancreatitis.

T-lymphocytes and their subsets were determined in three patients with classical Kaposi's sarcoma. A reduction of pan-T-lymphocytes, including both a reduction of T-helper and T-suppressor cells, was found in one patient. None of the patients showed a reduction of the T-helper/T-suppressor cell ratio, as described in the lymphadenopathic disseminated type of Kaposi's sarcoma. Intradermal testing with tuberculin and trichophytin gave no indication of humoral immunodeficiency or anergy. In addition to electron beam irradiation, one female patient was treated with rIFN-alpha-interferon, 6 X 10(6) IU 3 times a week over a period of 3 months, and 18 X 10(6) IU 3 times weekly over 1 month. Under this therapy, irradiated as well as nonirradiated lesions become flatter, reduced in size and faded. The number of T-suppressor cells diminished.

Two children are presented with Hodgkin's disease and a mediastinal mass which did not respond to polychemotherapy and radiation therapy and proved to be a benign thymic cyst. The coincidence of Hodgkin's disease with a benign thymic cyst has only been reported once before [11]. The diagnostic and therapeutic implications are discussed.