Twenty-five patients with peripheral T-cell lymphoma (PTCL) were analyzed to assess clinicopathologic features of the disease and were classified according to the newly proposed histologic classification for T-cell lymphomas to study the correlation between histology and survival, if any. Histologic diagnoses were: Lennert's, 1; T-zone, 1; pleomorphic, small, 1; pleomorphic, large, 1; angiocentric, 4; pleomorphic, medium, 5; immunoblastic, 5; angioimmunoblastic, 7. Patients with PTCL were noted to be endowed with clinicopathologic factors known to adversely affect survival, such as advanced stage (92%), B symptoms (56%), liver involvement (56%), multinodal disease (52%), elevated serum LDH (48%), and so on. Response to therapy was poor. CR was obtained in 30% of patients with advanced disease with a 50% relapse rate. Median survival for the 25 patients was 13 months. No correlation was found between morphologic subtypes and survival.

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Seven out of 16 patients with primary focal segmental glomerulosderosis (FGS) did not showed the nephrotic syndrome throughout their clinical courses, and then patients with FGS could be divided into the two groups, nephrotic FGS group (NS-G) and non-nephrotic one (NO-G). The clinicopathologic findings of NS-G and NO-G were compared retrospectively to define the pathogenesis of the glomerular disease in FGS. No significant differences were found between the groups except for proteinuria and serum total protein or albumin, and it was impossible to distinguish the two groups only histologically. In addition, electron microscopic study revealed that the glomerular epithelial cell was altered more than the endothelial and mesangial cells in both groups, resulting in vacuolization, foot process fusion, and detachment, and the striking fact that the glomerular epithelial vacuoles consisted mainly of the dilated rough endoplasmic reticulums (RERs). The speculation is drawn that in FGS the glomerular epithelial cell is firstly damaged by unknown factor(s), manifested functionally proteinuria and/or hematuria, morphologically dilatation of RER, foot process fusion, detachment, and eventually segmental sclerosis and hyalinosis.

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The mechanism which produces marrow failure in idiopathic aplastic anemia is still unknown. Recent investigations have suggested the crucial role of NK cells in the regulation of normal hematopoiesis. In this study, the cytotoxic activity of mononuclear cells from human bone marrow and peripheral blood was examined against three NK-sensitive target cell lines in 15 patients with aplastic anemia as well as 21 normal subjects. Marrow mononuclear cells from aplastic anemia demonstrated a high cytotoxicity comparable to peripheral blood NK cells to these target cells. Neither large granular lymphocytes nor the cells expressing known NK cell surface phenotypes increased in aplastic marrow cell elements. The aplastic marrow cells showed strong killing activity rather than binding at single cell assay. They consisted of non-adherent and adherent cell population in plastic adherence and were unresponsive to IFN treatment. The existence of cytotoxic cells with high NK-like activity maybe responsible for the mechanism of marrow failure in aplastic anemia.

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Recent studies of human monoclonal lupus autoantibodies have revealed that some of them are cross-reactive with platelets. To clarify further the bivalendes in anti-DNA and anti-platelet autoantibodies, we established nine human B cell lines producing monoclonal anti-platelet antibodies from patients with idiopathic thrombocytopenic purpura by Epstein-Barr virus transformation and studied the DNA-binding properties of these antibodies. Three of the antibodies showed cross-reactivity with single-stranded DNA (ssDNA), as determined by enzyme-linked immunosorbent assay and one of them was also found to bind to the synthetic polynucleotides; poly (dT) and poly (I), as well as to ssDNA. These findings suggest that cross-reaction between anti-DNA and anti-platelet antibodies is based on overlaps in specificity existing as common part of both autoantibodies.

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We studied the frequency of ST-T changes and ischemic heart disease (IHD) in prednisolone (PSL)-treated systemic lupus erythematosus (SLE) patients and compared them with the age-matched control of rheumatoid arthritis patients not receiving PSL. Twenty-five (38%) of the 65 SLE patients revealed ST-T changes as ST elevation (4%), ST depression (36%) and T wave flattening or inversion (60%). Among the control patients 4 (10%) had T wave flattening or inversion. The frequencies of ST-T changes in patients receiving total PSL dose of upto 5g and greater than 5g were 23% and 48%, respectively. Four patients developed IHD at an unusually young age during remission of SLE while receiving low dose of PSL and 2 of them later died of myocardial infarction (MI). The latter 2 patients had received PSL pulse therapy prior to MI. Regular ECG check up for SLE patients while they are on low dose PSL or pulse therapy may help reveal early ECG abnormalities and thus detect and treat one of the major risks of long-term effects of corticosteroid therapy.

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We experienced a case of malignant lymphoma with gastric and ileo-caecal lesions. The case was 80 year-old Japanese female with a chief complaint of anorexia. The patient had gross hematemesis on the 4th day of admission. By emergency gastroendoscopy, multiple small gastric ulcers which have sharp marginal prominences were observed. Pulsating arterial bleeding was observed and we successfully achieved hemostasis by topical injection of pure ethanol under gastroendoscopy. Biopsy specimen of that lesion showed it was malignant lymphoma.

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A 52-year-old female patient with massive ascites due to lupus peritonitis is described. Skin biopsy specimens revealed typical features of systemic lupus erythematosus (SLE) in light microscopic and immunofluorescent examinations. Immune-complexes, antinuclear antibody and hypo-complementemia were detected in the peritoneal fluid. The massive ascites responded dramatically to steroid pulse therapy. The levels of circulating immune-complexes, anti-nuclear antibody and complement in sera were also improved after such therapy. It was suggested that steroid pulse therapy may be useful for massive ascites due to lupus peritonitis.

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Probucol administration of 4 weeks produced torsades de pointes associated with exacerbated QT interval prolongation in a 36-year-old woman with Romano-Ward syndrome. With discontinuance of probucol, the QT interval corrected for rate shortened from 620 msec to 500 msec and ventricular ectopic beats disappeared completely. Although probucol is known to prolong the QT interval, associated ventricular tachyarrhythmia has not been reported in humans as yet. This case suggests that one should be very careful in the administration of probucol to patients with long baseline QT intervals.

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A 55-year-old female of Cushing's disease associated with multiple myeloma is reported. The association of the two diseases has not been previously noted. Elevated cortisol level was controlled successfully by low doses of adrenocorticolytic agent, o, p'-DDD. However, the exacerbation of multiple myeloma was found to accompany the serum cortisol normalization. Previous reports have shown the good efficacy of combination therapy with melphalan and prednisolone for multiple myeloma. It was suggested that the decrease of endogenous cortisol level might exacerbate the patient's multiple myeloma.

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A 29-year-old woman developed hypopituitarism following removal of a pituitary chromophobe adenoma, and this was complicated by type V hyperlipidemia and obesity.

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A 69-year-old female was treated for hyperthyroidism and hypertension. In August 1984, she suddenly began suffering from polyuria and polydipsia. In October, she exhibited fever, headache, vertigo, and poor appetite, probably due to pituitary apoplexy. Her endocrine function was normal, except for partial diabetes inspidus. A contrast-enhanced CT brain scan revealed a pituitary adenoma with a ring-enhanced outer edge and a central low-density area. The MRI scan also indicated cystic adenoma. A CT scan examination repeated 6 months later showed an empty sella with a markedly decreased pituitary adenoma. This case report demonstrates that some empty sella are the final result of pituitary adenoma bleeding or infarction.

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Although polypectomy by high frequency electric surgery through the use of endoscopy has been widely applied to polypoid lesions in digestive canals, there have been very few reports of the treatment of bronchial lesions with this procedure. Recently, we have been successful in performing polypectomy without any complications using high-frequency electric surgery through a flexible bronchoscope in a patient with a benign bronchial polyp. Bronchial polypectomy by electric surgery has the advantages of preventing bleeding and providing large specimens for histological examination, compared with conventional methods, i.e. for forceps or laser methods.

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A case of heterogeneous acute lymphoblastic leukemia of the Burkitt type (L3-ALL) is reported. A 58-year-old woman was admitted to our hospital with complaints of palpitation and shortness of breath in December 1988. On admission, the peripheral blood showed severe pancytopenia. The bone marrow was hypercellular with abnormal lymphoblasts; the cytoplasm was moderately abundant and intensely basophilic with prominent vacuoles. A diagnosis of L3-ALL was made. Cytogenetic analysis of bone marrowcells revealed a karyotype of 46, XX. The leukemic blasts were HLA-DR⁺, CD10⁺, CD19⁺ and CD20⁺, but no surface or cytoplasmic immunoglobulins were detected. This is the first report in Japan of a case of L3-ALL without chromosome abnormality and lacking surface and cytoplasmic immunoglobulins.

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Prolonged administration of diphenylhydantoin (DPH) has been implicated as a possible etiologic factor in immunological aberrations and lymphoproliferative disorders. Diphenylhydantoin may account for the increase in susceptibility to lymphoproliferative diseases, as a result of its immunosuppressive effect. We report a case of amyloidosis with monoclonal gammopathy which developed during DPH treatment, without multiple myelomaor lymphoproliferative disorders. The association between DPH and monoclonal gammopathy is very rare, and such a case of amyloidosis associated with DPH has not been reported previously. DPH, however, may have played a role in the development of monoclonal gammopathy, which was the precursor of amyloid protein.

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A 57-year-old female was admitted to our hospital with general lassitude, loss of appetite, nausea, upper abdominal pain, thirst, polydipsia and polyuria. On admission, she had an asymmetrical pear-shaped tumor in the right supraclavicular region and severe hypercalcemia. Plasma C-PTH was elevated to 22.72ng/ml. Plasma calcitonin was also elevated to 336 pg/ml. She died of respiratory and cardiac failure of two weeks after admission without any positive response to the treatment, including hemodialysis. Pathohistologically, the tumor was a parathyroid adenoma. The concentrations of C-PTH, intact PTH and calcitonin in the tumor tissue were markedly high: 4.56 μg/g wet, 13.9 ng/g wet and 50.7 ng/g wet, respectively. Immunohistologically, the tumor cells and the fibrous stroma were stained strongly positive to rabbit anti-human calcitonin antibody and rabbit anti-human N-PTH antibody by indirect immunoperoxidase staining. Calcitonin-producing tumors, except for medullary thyroid carcinoma are rarely reported. To our knowledge, this is the first report of such a calcitonin-producing parathyroid adenoma associated with primary hyperparathyroidism.

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Three cases are reported who have short runs of atrial premature contractions (APCs) induced by swallowing and cough. The occurrence of the APCs were affected by autonomic nervous system in all cases. The precipitating factor is considered to be the increase of vagal tone in case 1, sympathetic tone in case 2 and both of them in case 3. The autonomic mechanism of swallowing-induced tachycardia could be divided into three categories and the treatment of these arrhythmias might be different in each group. Pharmacological autonomic blockade with atropine and propranolol is useful to reveal the underlying autonomic mechanisms.

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A 46-year-old woman was admitted to the hospital because of dyspnea and palpitation. Physical examination showed severe anemia and cardiac distress. Telangiectases were found in the conjunctivas, lips and oral mucosa. Chest roentogenogram showed an enlarged cardiac silhoutte and multiple coin lesions in both lower lung fields. We diagnosed her as Rendu-Osler-Weber disease (ROW). The angiographic studies showed hemangioma in the foramen of Monro, bilateral multiple pulmonary arteriovenous fistulas, and hepatic arteriovenous fistula with a large distortion of the proper hepatic artery. Although associated vascular dysplasias have been described in patients with ROW, there is no reports on ROW in which multiple vascular abnormalities concomitantly existed such as the present case. In 163 detailed cases of ROW in Japan, the frequency of vascular lesions was summarized, and this result showed that the vascular lesions of the lung were more frequent than that of the liver or brain, in comparison with countries in Europe and America. It is very important for diagnosis and its prognosis to perform a detailed angiographic examinations and to demonstrate the precise sites of the vascular dysplasias in patients with ROW.

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