[An Autopsy Case of Primary Myelofibrosis with Skin Tumors Terminating in Leukemic Transformation (author's transl)].
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Biomedical subjects
Publications and source records attributed to Y Hamashima.
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Seventeen autopsy cases with occlusion of the circle of Willis were examined clinicopathologically. These patients ranged in age from 8 years to 64 years and consisted of 6 males and 11 females. The following results were obtained. 1. Fresh and massive intracerebral hemorrhage was confirmed in 13 of 17 patients and cerebral infarct in 4 of 17 patients. 2. Among these 13 patients, massive hemorrhage was found in basal ganglia, thalamus and hypothalamus of 9 patients, and in thalamus, cerebral peduncle and midbrain of 4 patients. 3. Rupture of the dilated muscular-type arteries was noted in the thalamus and basal ganglia in 2 of 13 patients. Ruptured arteries with organization of the lumen were found in the old hemorrhagic foci in one of them. 4. Overgrown and dilated arteries, branching off from the circle of Willis, were confirmed in 11 patients. These arteries consisted of "perforating" arteries well developed as collateral circulation. 5. No ruture in these arteries of the subarachnoidal spaces was found in 11 patients. In one of them, a small saccular aneurysm was found. These findings strongly suggest that in patients with occlusion of the circle of Willis intracerebral rupture of overgrown "perforating" arteries as collateral circulation may be the main cause of intracranial hemorrhage and intracerebral hemorrhage due to rupture of the arteries may be repeated.
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Twenty-seven skin biopsies were obtained from the exanthemata of patients in the acute stage of the mucocutaneous lymph node syndrome (MCLS). The three vascular systems of different caliber size--the intrapapillary capillary loops (ICL), the superficial arteriolar or venular plexus (SAP, SVP) and the small subcutaneous vessels--were examined to investigate the characteristics of the vasculitis in MCLS and differentiate it from infantile polyarteritis nodosa (IPN). Significant papillary edema and dilatation of ICL, SAP and SVP were observed on the 4th day after the onset of the illness, and then gradually decreased. In the subcutaneous regions, vasculitis began with endothelial necrosis, and subendothelial edema and degenerative changes in the muscle cells followed. These changes in the small subcutaneous vessels were observed for a longer period than in the ICL, SAP and SVP. Moderate mononuclear cell infiltrations were observed. Both arteries and veins were affected.
Clinicopathologic study of the conduction systems was done on ten hearts obtained at autopsy from patients with Kawasaki's disease. The pathologic lesions were discovered in the atrioventricular (AV) conduction system in nine out of 10 cases and in five of eight cases in the sinoatrial (SA) conduction system. The lesions of the AV conduction system were classified according to duration of illness at death. Eearly acute state (0 to 9 days) was characterized by inflammation with cellinfiltration and edema without coronary stenosis. The most severe acute stage (21 to 31 days) was characterized by severe compression of conduction cells without the diffuse necrosis due to severe perivascular edema and cell infiltration. In the end stage (48 days to 7 months), old changes with perivascular fibrosis and fatty infiltration without considerable loss of the conduction cells were noted. Coagulation necrosis of the conduction cells was not evident despite the severe coronary stenosis. Lesions in the conduction system are therefore acute and inflammatory. Pathology and electrocardiogram showed a good correlation in seven of eight cases. PQ prolongation was a sensitive indicator of acute inflammation of the AV conduction system in the present study.
Rickettsia-like bodies were reportedly found in biopsies of the skin and lymph nodes from half the number of patients with Kawasaki's disease. In our present work, these microbodies were isolated from the peripheral whole blood of a patient with Kawasaki's disease (MLNS) through passage of guinea pigs and yolk sac culture. However, the isolated strains disappeared spontaneously during their stores at -80 degrees C for two weeks.
NBT (Nitroblue Tetrazolium) test was performed in 17 patients with Kawasaki disease to examine the function of phagocytosis and intra-cellular killing of neutrophils. The value was high compared to other pediatric patients. Activation with Proteus OX-2 antigen before NBF test showed a significant higher level than other proteus antigens, which correspond in serum level. With previous electronmicroscopic observation of rickettsia-like body in biopsy specimen, these findings suggest the existence of an agent in Kawasaki disease which shares antigenicity with Proteus OX-2.
Pathological findings of coronary heart disease were compared between patients with lung cancer and with cancer in the other organs in male subjects over 55 years of age. Myocardial infarction was found in 13 of 58 subjects with lung cancer and in 5 of 79 subjects with cancer in the other organs (P less than 0.02). The subjects with severe stenoses of major coronary arteries were more frequent in lung cancer group (13 of 58) than in cancer group in the other organs (4 of 79 subjects: p less than 0.01). In 12 of 13 subjects with lung cancer and myocardial infarction the infarction seemed to occur due to coronary stenosis. The subjects with lung cancer had significant increase of heart weight in comparison with subjects with cancer in the other organs.
Pathologic studies were done on 20 hearts of patients who had typical clinical signs and symptoms of Kawasaki disease. The cardiac lesions were classified according to the duration of illness at the time of death. Stage I (zero to nine days) was characterized by acute perivasculitis and vasculitis of the microvessels (arterioles, capillaries, and venules) and small arteries, and acute perivasculitis and endarteritis of the three major coronary arteries (MCAs). Pericarditis, myocarditis, inflammation of the atrioventricular conduction system, and endocarditis with valvulitis were also present. Stage II (12 to 25 days) was characterized by panvasculitis of the MCAs and aneurysm with thrombus in the stems. Myocarditis, coagulation necrosis, lesion of the conduction system, pericarditis, and endocarditis with valvulitis were also present. In stage III (28 to 31 days), granulation of the MCAs and disappearance of inflammation in the microvessels were noted. Patients in stage IV (40 days to 4 years) had scarring with severe stenosis in the MCAs. Fibrosis of the myocardium, coagulation necrosis, lesions of the conduction system, and endocardial fibroelastosis were also present. The features observed revealed Kawasaki disease to be acute and inflammatory. The angiitis begins in the microvessels and fibrinoid necrosis of the media is rare. The disease is one with a pathologic pattern previously unknown.
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Chemical sympathectomy induced by 6-hydroxydopamine (6-OHDA) suppressed the secondary immune response of mice to a T-cell (thymus derived lymphocyte) dependent antigen, sheep red blood cells (SRBC). Treatment with 6-OHDA on the day of the secondary injection of SRBC resulted in depression of hemagglutinin titers to the antigen, while treatment with 6-OHDA on the day of the primary injection of SRBC had no effect upon the secondary response to the antigen. In addition, 6-OHDA treatment did not suppress the primary immune response to a T-cell independent antigen, Escherichia coli lipopolysaccharide (LPS). These results suggest that it is the T-cells which are mainly affected by chemical sympathectomy. Significant non-specific toxicity was not observed with 6-OHDA 100mg/kg, the dose of which suppressed the primary and the secondary immune response to SRBC.
The effects of general sympathectomy with 6-hydroxydopamine (6-OHDA) on antibody production to sheep red cells (SRBC) were studied in mice. Intraperitoneal administration of 6-OHDA in a dose of 1 to 300mg/kg resulted in a significant decrease in hemagglutinin titer and number of direct plaque-forming cells which were observed only in the early period of the primary immune response. Following treatment with 6-OHDA 10mg/kg i.p., the noradrenaline content in murine spleen was significantly reduced from 63 to 42% of control value between 2 and 10 days after injection indicating that chemical sympathectomy suppresses the primary immune response.
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