Thirty-five patients with Philadelphia chromosome (Ph¹)-positive chronic myelogenous leukemia (CML) were classified on the basis of the fusion pattern of bcr-abl mRNA determined by the reverse-transcriptase-polymerase chain reaction (RT-PCR) method. Semiquantitative assay of the bcr exon 2/abl exon 2 fused mRNA (b2-a2) and bcr exon 3/abl exon 2 fused mRNA (b3-a2) resulted in 21 patients showing b3-a2 type mRNA, seven showing b2-a2 type and seven showing coexpression. Quantification of the autoradiographic signals of amplified products was estimated using an MCID image analysis system. The relative intensity was defined as the ratio of bcr-abl signal to that of B-actin. The relationship between the semiquantified bcr-abl mRNA and the platelet/megakaryocyte counts was analyzed. A possible correlation was found between the semiquantified b3-a2 type mRNA and the platelet (p<.05, N=28) and megakaryocyte (p<.05, N=13) counts of these patients. This finding suggests the possibility that b3-a2 mRNA may affect the thrombopoietic activity in Ph¹-positive CML in a dose-response manner.
(Internal Medicine 33:189-192, 1994)

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Mean (±SD) serum erythropoietin (EPO) levels were 18.6±5.6 mU/ml in 180 normal Japanese subjects. Serum EPO levels were elevated with a negative correlation on a log scale (r=-0.864, P<0.005) to hematocrit (Ht) values in anemic patients not associated with rheumatoid arthritis (RA) or chronic renal failure (CRF). Serum EPO levels in patients with RA (31.6±16.4 mU/ml) were relatively lower than those in normal subjects and anemic patients without RA or CRF when matched for comparative Ht values. Seven anemic patients with RA were treated by daily subcutaneous (sc) injection of recombinant EPO (rEPO, 500-1, 000 U/day) for 4 weeks. The patients had initial Ht values of 25.1% or less and maintained stable clinical status. The treatment with rEPO raised serum EPO levels (53.8±15.2 mU/ml, P<0.05), which resulted in an increase in Ht values (more than 3%) in 6 out of 7 patients with RA. The mean (±SD) Ht values at the end of the treatment with rEPO (500-1, 000 U/day) were greater than those before the treatment in the 7 patients with RA (28.5±4.6 vs. 22.7±2.5%, P<0.05). These findings suggest that chronic anemia associated with RA may be corrected by daily sc injection of a small dose of rEPO.
(Internal Medicine 33:193-197, 1994)

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A case control study was performed with 142 leukemia patients and 284 controls matched for age and sex. Occupation, birth order, past medical history, and drinking and smoking habits were compared in these two groups. Persons born first or fourth were found to have a higher incidence of leukemia. History of a fracture was one of the risk factors for acute leukemia, and a history of gastroduodenal ulcer was a risk factor for chronic leukemia. This may suggest that extensive exposure to X-rays in diagnosis and treatment is a risk factor for leukemia. There was a significant dose-response relationship between the amount of smoking and the incidence of acute nonlymphocytic leukemia, but not between the amount of alcohol consumption and the incidence of leukemia. Thus, smoking was one of the risk factors for acute leukemia.
(Internal Medicine 33:198-203, 1994)

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We studied the feasibility and adverse effects of transpulmonary contrast echocardiography using sonicated albumin in 6 dogs. Left heart contrast was observed by two-dimensional echocardiography, monitoring left ventricular pressure, its first derivative (dP/dt) and arterial oxygen saturation. The left heart was adequately opacified in 48 of 55 injections of various intravenous doses of sonicated albumin. Left ventricular myocardial opacification was faintly observed in only 1 injection. After injections of a large dose (0.5 ml/kg), left ventricular systolic pressure decreased, maximal positive dP/dt decreased and negative dP/dt increased, slightly. Hemodynamic parameters showed no significant changes with the other doses. Arterial oxygen saturation tended to decrease after injection, which was most remarkable with a large dose injection. In conclusion, although a large dose of intravenous sonicated albumin may opacify not only the left heart cavity but the ventricular myocardium, it deteriorates hemodynamic parameters and oxygen exchange in the lung.
(Internal Medicine 33: 204-209, 1994)

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In addition to electrocardiogram (ECG), analysis of creatinine phosphokinase (CPK), lactate dehydrogenase (LDH) and their isoenzymes is commonly employed to diagnose acute myocardial infarction (AMI). The aim of this study was to observe the efficiency of isomers of CPK for early diagnosis of AMI. ECG, though useful in many ways, is not 100% sensitive. The drawback involved with CPK and LDH isoenzymes is the time lag factor in diagnosing AMI early enough for interventional therapy. Recently the subunits of these isoenzymes, namely MM and now the MB isomers are being tested for their effectiveness in the early diagnosis of AMI. In our experience MB isomers were found to be accurate markers in the early diagnosis of AMI. They were especially valuable in diagnosing myocardial infarction not detectable by ECG in cases such as non-Q AMI, cardiomyopathy, and bundle branch block among other such cases.
(Internal Medicine 33: 210-215, 1994)

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This report describes a case of light chain deposition disease (LCDD) with unusual findings of fibrillar structures in the deposits and marked calcification in several organs. A forty-year-old man was initially diagnosed with LCDD in 1987, and died of sepsis three and one-half-years later. Histological examination of autopsy specimens demonstrated eosinophilic amorphous materials, which differed from amyloid, in vessel walls or around parenchymal cells in almost every organ examined. Ultrastructurally, in addition to granular deposits, fibrillar structures were also seen in the deposits. Marked calcification was present in the myocardium, skeletal muscles, adrenal glands and arteries.
(Internal Medicine 33: 216-221, 1994)

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A case of muscular sarcoidosis accompanied by severe hypercalcemia (serum calcium 15.5 mg/dl), renal insufficiency and renal calcification is reported. Sarcoid granulomas were found in the biopsy specimens of the lung and the muscle. The administration of prednisolone effectively improved not only muscle weakness but also hypercalcemia, renal insufficiency and renal calcification. This is a rare case of sarcoidosis in which renal calcification remitted after treatment with prednisolone. It is thus suggested that prednisolone treatment should be considered not only for hypercalcemia, but also for renal insufficiency caused by renal calcification in patients with sarcoidosis.
(Internal Medicine 33: 222-225, 1994)

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A 46-year-old man was admitted to our hospital with cough and dyspnea on exertion. A chest X-ray film showed diffuse interstitial shadows. He had hyperpigmentation forming a network pattern around his neck and dystrophy of the fingernails and toenails, and was diagnosed as having dyskeratosis congenita. Histological examination of the lung specimen obtained from the left S⁴b by open lung biopsy revealed usual interstitial pneumonia pattern with neither asbestos bodies nor silicotic nodules. Taken together with previously published findings, pulmonary involvement is considered to be an important complication of dyskeratosis congenita.
(Internal Medicine 33: 226-230, 1994)

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Nephrogenic diabetes insipidus associated with ureteral obstruction is rare. We report a case of nephrogenic diabetes insipidus associated with ureteral obstruction caused by ileal leiomyosarcoma in a 32-year-old man. The treatment with trichlorothiazide and diclofenac sodium reduced urine output from 8 L/day to 4 L/day. Six months after nephrostomy, urine output decreased to 2.5 L/day without any drug administration. This case suggests that ureteral obstruction may cause an increase in urine output to 8 L/day and that surgical treatment for ureteral obstruction is effective in reducing urine output in nephrogenic diabetes insipidus patients with ureteral obstruction.
(Internal Medicine 33: 231-233, 1994)

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A 58-year-old woman developed diabetic ketoacidosis and emphysematous pyelonephritis caused by Escherichia coli. She was successfully treated with nephrectomy, antibiotics, and recombinant human granulocyte colony-stimulating factor (rhG-CSF). RhG-CSF therapy may be an effective adjunct for diabetic patients with severe infection, even when neutropenia is not present.
(Internal Medicine 33: 234-236, 1994)

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A case of idiopathic plasmacytic lymphadenopathy with hyperimmunoglobulinemia is reported. A 71-year-old man was admitted to the hospital because of an abnormal shadow on chest roentgenogram. Chest X-ray taken on admission showed remarkable diffuse infiltration and pleural thickening. Laboratory examinations revealed an elevated total protein level of 10.1 g/dl, and a gammaglobulin level of 7.0 g/dl including 6, 790 mg/dl IgG. Mediastinal lymphadenopathy was observed on a chest CT. The patient underwent open lung biopsy. Heavy infiltration of lymphocytes and plasma cells were seen in the moderately fibrotic pulmonary interstitium. LIP was diagnosed. Lymph node biopsy was also performed. Follicular hyperplasia with prominent germinal centers and plasma cell proliferation in the interfollicular area were seen. Treatment with prednisolone resulted in an improvement in the chest X-ray findings, as well as a diminished polyclonal hypergammaglobulin level.
(Internal Medicine 33: 237-241, 1994)

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A family with Hemoglobin Bethesda is reported. A 23-year-old man was hospitalized for the evaluation of polycythemia. Analysis of hemoglobin using high pressure liquid chromatography showed the presence of hemoglobinopathy. Separation of globin into α and β chains revealed approximately 50% of the β chain to be abnormal. Analysis of the DNA sequence of the β chain gene identified Hb Bethesda. The family study disclosed that his father and sister also had the same hemoglobinopathy. This case is the first report of Hb Bethesda in Japan.
(Internal Medicine 33: 242-247, 1994)

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A 53-year-old patient with Guillain-Barré syndrome preceded by herpes simplex virus infection developed bilateral tonic pupils with light-near dissociation. Pharmacological tests for pupils suggested postganglionic involvement of the parasympathetic and sympathetic nerves. A demyelinating process of peripheral autonomic nerves was suspected to be the cause of the tonic pupils and autonomic dysfunction.
(Internal Medicine 33: 248-251, 1994)

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A 55-year-old man developed multiple erythematous skin lesions after surgery for acoustic neurinoma. Necrosis and detachment of the epidermis developed and a diagnosis of toxic epidermal necrolysis (TEN) was made. Progressive dyspnea was evident two days after the onset of the skin lesions. Chest X-ray revealed diffuse interstitial shadows in both lung fields. On bronchoscopic examination, erosion, vesicle formation, necrosis and desquamation of the epidermis were seen in the tracheobronchial mucosa. Therapy, including prednisolone, improved the skin lesions as well as the chest X-ray and bronchoscopic findings. Tracheobronchial and pulmonary lesions were followed before, during and after treatment of TEN.(Internal Medicine 33: 252-255, 1994)

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