Multiple myeloma associated with amyloidosis and t(1;20)(q21;q11) translocation.

A 59-year-old woman with nephrotic syndrome was diagnosed as having primary amyloidosis based on the detection of amyloid deposition (AL-protein) in the esophagus and kidneys. Bone marrow aspirate showed plasmocytic proliferation, leading to a diagnosis of multiple myeloma (IgG lambda-type). In addition, a very rare translocation t(1; 20) (q21; q11) was seen by chromosomal analysis of both the bone marrow and peripheral blood.

Acute autonomic neuropathy associated with a high serum bradykinin level and positive anti-nuclear and anti-DNA antibodies titers.

A hypotensive 37-year-old man developed the problems of impotence, lack of sweating, orthostatic hypotension, and convulsive syncopal attack. His blood pressure fell to 53 mmHg systolic following bed-tilting from 30 to 60 degrees, but his heart rate remained constant which indicated a diagnosis of acute autonomic neuropathy. With the tilting test, a decrease in serum catecholamine levels and an increase in bradykinin levels were observed. Four months after admission, anti-nuclear antibody, anti-DNA antibody, and the LE test became positive. The acute autonomic neuropathy appeared to be associated with SLE, and the hyperbradykinism, consequent on orthostatic hypotension.

[Evaluation of increasing digital blood flow during early period of air-cooled cold test].

The present study was performed to elucidate peripheral hemodynamic changes, especially, digital blood flow, caused by an air-cooled cold test. Experiments were carried out by placing the subject's left hand in a box that was kept at a temperature of about 18 degrees C by air-cooling. At the same time, the digital blood flow, digital blood pressure, compliances of the peripheral resistance and capacitance vessels were measured. These parameters were measured on the left forefinger of the cooled side, and also on the opposite side according to Kato's method at 3 points, 1) at normal condition (before cooling stated). 2) 30 seconds after the cooling began and 3) 10 minutes after the cooling began. The following results were obtained; 1) The systemic blood pressure, digital blood pressure and heart rate showed no statistically significant differences in measurements taken at the above three stages. 2) The mean value of the digital blood flow was found to have increased after 30 seconds, and to have decreased after 10 minutes of cooling. Statistically, significant differences were noted at the above three stages. 3) The mean value of the peripheral vascular resistance was found to have increased after 30 seconds, and to have decreased after 10 minutes. 4) Compliances of the peripheral resistance vessel and capacitance vessel showed no significant changes on either side except between normal condition and after 10 minutes of cooling.

A case of Turner syndrome with the karyotype of 45,X/46,X,i(Xq) associated with acute monocytic leukemia.

An infertile 37-year-old woman was diagnosed as having acute monocytic leukemia (AMoL) (FAB classification; M5b). In addition, a diagnosis of infertile Turner syndrome was made, based on the presence of the ovarian dysplasia, abnormal physical features (short stature, lack of pubic hair, shield-like chest, etc.), and low urinary estrogen excretion with high plasma gonadotropin level. Karyotypes in the peripheral blood and bone marrow cells were mosaic 45,X and 46,X,i(Xq): isochromosome Xq, which were consistent with infertile Turner syndrome. No further chromosomal abnormalities were found during the course of her treatment for leukemia. This is the first report of the combination of Turner syndrome and AMoL. However, this patient did not have any of the other autosomal chromosomal abnormalities which are common in acute non-lymphocytic leukemias.

Total body water, total exchangeable sodium and potassium in patients with convalescent acute myocardial infarction one to two months after onset.

Total exchangeable sodium (Nae), potassium (Ke), and total body water (TBW) were measured by the multiple isotope dilution method, in 10 healthy subjects (normal), 10 patients with congestive heart failure (CHF), and 47 patients with acute myocardial infarction (AMI), 1-2 months after onset. According to Killip's classification, 29 patients with AMI were classified as class I, and 18 patients were classified as class II and III (referred to as class II & III). No differences were found in plasma and urine sodium and potassium concentrations. By the multiple isotope dilution method, significant elevations in Nae/BSA (body surface area) were observed in the following order: normal, class I, class II & III and patients with CHF. Compared with normal subjects, Nae/BSA and Nae/Ke were elevated in class I patients. Elevations of Nae/Ke and TBW/BSA in both class II & III patients with AMI and patients with CHF indicated severe cardiac impairment. Both Nae/BSA (p = -0.60) and Ke/BSA (p = 0.71) had negative and positive correlations with the left ventricular ejection fractions (EF) measured by catheterization in 20 patients with AMI. This indicates a major sodium and water retention mechanism due to impaired cardiac function in AMI. It is worth noting that conspicuous abnormalities in body fluid compositions, particularly in class I patients with AMI as well as class II & III, remained despite no evidence of cardiac failure.

[Mandibular tumor with spicule formation in multiple myeloma].

A 40-year-old male complained of swelling of the right mandible. It was a hard bone tumor which was 70 x 50 x 45 mm in size without tenderness. In serum, the level of IgG was 5814 mg/dl, IgA 95 mg/dl and IgM 8 mg/dl with monoclonal increasing of gamma and lambda-chain. No Bence Jones protein was detected. In a bone marrow aspirate atypical plasma cells were 7.3% in nucleated cells in slightly hypocellular marrow. X-ray examination disclosed a spicule formation surrounding the osteolytic focus in the mandible. In addition, a osteolytic tumor without spicule formation was found in a rib. The histological examination of biopsy specimen obtained from the mandibular tumor revealed that atypical plasma cells infiltrated the fibrous tissue with new bone formation. Spicule formation in the bone lesion of multiple myeloma is unusual.

Cortisol and its metabolites in the plasma and urine in Cushing's syndrome with chronic renal failure (CRF), compared to Cushing's syndrome without CRF.

A 48-year-old man was admitted for treatment of Cushing's syndrome due to right adrenal adenoma, associated with chronic renal failure (CRF) with a blood urea nitrogen level of 64.2 and serum creatinine level of 3.9 mg/dl. After removal of the adrenal adenoma, the CRF deteriorated with progressive symptoms of anorexia, vomiting and hypertension, and the patient was placed on hemodialysis. Prior to adrenalectomy, the 17 OHCS and 17 KGS in the urine were not so high. However, the urinary 17 KS was high with an elevated 11-oxy fraction. In comparison with 2 patients suffering from adrenal Cushing's syndrome with normal renal function, there were no large accumulated quantities of glucuronic conjugated and unconjugated metabolites in the plasma of the CRF Cushing's syndrome, with confirmation ascribable to the radioimmunoassayable cross-reactivity of the cortisol antiserum used in the radioimmunoassay kit. In the Cushing's syndrome with CRF, almost all the cortisol, which was hypersecreted from the adenoma, was presumed to be converted to the 11-oxy fraction of 17 KS, possibly by activation of hepatic enzymes.

A case of hyperthyroidism caused by the syndrome of inappropriate secretion of thyroid stimulating hormone: association of primary hypergonadotropic hypogonadism.

A 49-year-old man with hyperthyroidism caused by the syndrome of inappropriate secretion of TSH (SITSH) is reported. A diagnosis of infertility and hypospermatogenesis due to primary hypergonadotropic hypogonadism had been made 10 years ago. Serum TSH levels were inappropriately elevated with thyrotoxic high levels of thyroid hormone in serum, but no tumorous mass was demonstrated in the pituitary gland. The non-tumorous pituitary resistance of TSH to thyroid hormonal suppression was confirmed by low levels of glycoprotein alpha-subunit and low alpha-subunit/TSH ratios in serum before and after the stimulation by TRH. In addition, the patient had elevated levels of LH and FSH in serum, and their exaggerated responses to LH-RH, although serum testosterone concentrations were normal, indicating primary hypogonadism. It is of interest that SITSH appeared in the hypogonadal patient who was preceded by the long standing hypersecretion of LH and FSH.

Cervical catecholamine-secreting paraganglioma in the pterygopalatine fossa.

A 25-year-old man was admitted for examination to determine the cause of hypertension. High levels of noradrenaline in plasma and urine were seen, suggesting that the patient had an adrenal or extra-adrenal pheochromocytoma. However, whole-body scintigraphy using the isotope of [131I] meta-iodo-benzylguanidine (131I-MIBG) failed to find the presence of a catecholamine-secreting tumor. The highest level of noradrenaline was detected in plasma obtained from the left jugular vein after selective venous collections through femoral catheterization. Both the computed tomography and cerebral angiography showed a hen-egg-sized tumor located in the left pterygopalatine. After surgical removal of the tumor, high blood pressure and the levels of noradrenaline in plasma and urine were significantly decreased. Histopathological diagnosis was paraganglioma (catecholamine-secreting paraganglioma). The patient with cervical catecholamine-secreting paraganglioma is the first case where the tumor was isolated and located in the pterygopalatine fossa.

Significant regression of the tumor combined with the high level of plasma cortisol by low-dose administration of O, p'-DDD in a case with Cushing's syndrome caused by adrenocortical carcinoma.

The effect of o, p'-DDD on a functioning adrenocortical carcinoma is described. A 52-year-old woman, who had a huge adrenocortical carcinoma and pulmonary metastasis, was treated by a low-dose administration of o, p'-DDD, which reduced the tumor size. The excretion of urinary 17-OHCS was decreased, whereas plasma cortisol was not decreased but rather increased. This was considered to be caused that o, p'-DDD functioned as a cytotoxic factor which did not improve the states of hyperkalemia, hyperglycemia and hypertension. This suggested that monitoring to measure multiple steroids in addition to plasma cortisol and urinary 17-OHCS was useful for recognizing the general conditions of patients with adrenocortical carcinoma receiving o, p'-DDD therapy.

Chromatographic elution profiles of angiotensin converting enzyme measured by the methods using different substrates in the plasma from normotensive and hypertensive subjects.

The angiotensin converting enzyme (ACE) activity in the plasma collected from normotensive and hypertensive patients was measured chromatographically by two different methods using hippuryl-histidyl-leucine (HHL-ACE) and synthetic angiotensin I (AI-ACE) as substrates. A single peak was observed in the elution profiles of HHL-ACE on both Sephadex G 150 column and DEAE Sephadex A 50 cellulose column chromatographies. A single peak of AI-ACE appeared on the gel-filtration. However, several peaks of AI-ACE were seen on DEAE Sephadex A 50 cellulose column chromatography, suggesting that there are various types of ACE which hydrolyze angiotensin I but not HHL.

[Effect of ACTH on plasma renin activity, aldosterone level and deoxycorticosterone level in humans (author's transl)].

Plasma aldosterone level (PAL) and deoxycorticosterone level (PDL) were determined in 5 subjects of normal sodium intake and 5 subjects of low sodium intake after the intramuscular administrations of ACTH (Upjohn, Cortrosyn) 40 IU, using radioligand methods. PAL and PDL reached their peaks at one hour after the administrations of ACTH, and then decreased in their normal ranges. Pretreated with the oral administrations of Dexamethasone 2 mg/day for 2 days, these subjects had, when ACTH were injected, such a similiar pattern in PAL and PDL as the peak of one hour and the consecutive falling without the pretreating of Dexamethasone. PAL and PDL of these subjects were not different between normal sodium intake and low sodium intake during the course of the experiment.