Fragmentation hemolysis in a patient with hypertrophic obstructive cardiomyopathy and mitral valve prolapse.

We encountered a 65-year-old female with hypertrophic obstructive cardiomyopathy and mitral valve prolapse who had infective endocarditis and hemolytic anemia. The infecting organism of endocarditis was group A streptococci. With regard to the etiology of the hemolytic anemia, fragmentation hemolysis was considered because fragmented red cells and elevated lactic dehydrogenase were observed. Haptoglobin was markedly decreased. Coombs' test, Ham's test and abnormal hemoglobin were negative. She had not had a hemolytic attack in the past. Ultrasonic cardiography showed asymmetrical septal hypertrophy, mitral valve prolapse and 285 mmHg of calculated pressure gradient in the left ventricle. Cardiac catheterization showed 115 mmHg of left intraventricular pressure gradient and mitral regurgitation (grade 2). Hemolysis was slightly improved after treatment with propranolol. Thus, fragmentation of the normal red cells seemed to be due to shear stress.

Normotensive glucocorticoid-suppressible hyperaldosteronism in adult.

A 40 year-old man was admitted to our hospital for detailed examination of hypokalemia (2.7 mEq/l). His blood pressure was normal. Metabolic alkalosis, ACTH dependent hyperaldosteronism (18 ng/dl) and over-response to synthetic ACTH were observed. Plasma renin activity, on the other hand, was within the normal range (1.7 ng/ml/hr). Serum potassium was normalized to 4.1 mEq/l and the responsiveness of the renin-angiotensin-aldosterone system was recovered after the administration of dexamethasone. These results led us to suggest that this case might be normotensive glucocorticoid-suppressible hyperaldosteronism. The etiology which was not associated with hypertension and low plasma renin activity has not been clarified but may be related to the shortness of duration of this disease. Our case was also afflicted with mild hypercortisolemia and excessive excretion of urinary 17-hydroxycorticosteroid and 17-ketosteroid which was suppressed by the administration of dexamethasone (2 mg/day). These findings may be related to hypersensitivity of the fascicular zone of the adrenal gland to ACTH.

Chronic myelomonocytic leukemia with polyneuropathy and IgA-paraprotein.

A 65-year-old woman had chronic myelomonocytic leukemia with peripheral neuropathy and IgA paraprotein with kappa type light chain. Plasma cells with "flaming" cytoplasma were seen in the bone marrow specimens. The findings suggest that chronic myelomonocytic leukemia may involve B-lymphocytes in the proliferative process and that it may be a clonal disease. Moreover, this case was associated with peripheral sensori-motor polyneuropathy. The disorder was responsive to prednisolone, as oral administration of prednisolone improved not only the peripheral polyneuropathy, but also the hematological findings. Serum vitamin B12 and lysozyme in the serum and urine were decreased by the steroid therapy. The administration of prednisolone is effective not only for polyneuropathy but also chronic myelomonocytic leukemia.

A case of calcitonin-producing parathyroid adenoma with primary hyperparathyroidism.

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 micrograms/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.

[Discrepancy in karyotypes between peripheral blood and bone marrow during blastic crisis in chronic myelocytic leukemia].

A 32-year-old female was diagnosed as having Ph1-positive chronic myelocytic leukemia (CML) on March 6, 1985. She was intermittently treated with busulfan or 6-mercaptopurine. Her regimen was changed on February 27, 1987 to interferon-alpha (HLBI, Sumitomo) because of leukocytosis (46,200/microliters) with basophilia (45%) and splenomegaly refractory to conventional therapy. She was admitted to our hospital on November 27, 1987 because of blastic crisis. Cytogenetic analysis on peripheral cells was repeated six times during the treatment with HLBI. The sixth analysis was done on bone marrow cells as well. Nineteen to 22 metaphases were analyzed by the trypsin G-banding method after short-term culture. Cytogenetic analysis of peripheral cells revealed 46, XX, Ph1 in 9% of metaphases and 47, XX, Ph1, +8, i(17q) in 91% on March 2, 1987, and 47, XX, Ph1, +8, i(17q) in 95.2% of metaphases and 48, XX, Ph1, +8, i(17q), +19 in 4.8% on December 11, 1987. Karyotypes of bone marrow cells on December 11, 1987 were 48, XX, Ph1, +8, +8, 4(17q) in 73.7% of metaphases and 47, XX, Ph1, +8, i(17q) in 26.3%. It was speculated that abnormal clones might have developed in other sites than bone marrow.

[Case of hepatocellular carcinoma with a marked reduction in the tumor size induced by PSK administration alone].

A 56-year-old man was admitted to our hospital because of right abdominal pain. The edge of the liver was felt 4.5 fingers breadth below the xiphoid process. AFP was 20100 ng/ml and CEA was 5.6 ng/ml. The chest X-ray indicated existence of lymphangitis and some nodular density suggesting lung metastasis in the both lower-lung fields. 99mTc-phytate liver scan showed a large defect along the antero-inferior margin of the right hepatic lobe, which revealed an abnormal uptake of 67Ga-citrate. Ultrasonograms demonstrated a solid mass, 8 X 9 cm, in the right lobe of the liver. A CT-scan of the abdomen also showed a large, rounded, low attenuation mass with central necrosis in the right hepatic lobe: the pancreas and the remaining retroperitoneal structures appeared normal. Following the administration of PSK alone, 3 g daily, for three months, a remarkable regression of both hepatomegaly and lung metastasis was observed. Liver scan, ultrasonograms and CT-scan showed a striking resolution of the intrahepatic mass except central necrosis. AFP decreased to 33.7 ng/ml and CEA was 8.2 ng/ml. After about one year, however, ultrasonogramms showed a newly growing solid mass, 3.5 X 3.5 cm, in the left lobe of the liver. A needle biopsy specimen was taken from the intrahepatic mass, and it was interpreted as hepatoma. He is now healthy.

[Diagnostic system for assessment of left ventricular function with 99mTc-albumin (author's transl)].

Diagnostic system is described that obtains a set of serial gated images (SGI) covering the entire cardiac cycles and left ventricular (LV) volume curve (VC) with high temporal resolution (10 msec). The system consists of two functional parts. The one, which is based upon an inexpensive modification of multiformat imaging device, yields SGI. The other, which is based on a minicomputer system, acquires data only from around about LV area and yields LVVC from ECG P wave without the reconstruction of these images. In as little as 5 min result, which is also corrected uniformity of gamma-camera, is given.