Acute megakaryoblastic leukemia with complex chromosomal aberrations

A case of acute megakaryoblastic leukemia with complex chromosomal aberrations is reported. A 63-year-old man was admitted to our hospital because of pancytopenia. Bone marrow aspiration resulted in a dry tap and biopsy showed hypoplastic marrow with fibrosis. Blast cells in the peripheral blood were identified as megakaryoblasts because they were positive for electron microscopic platelet peroxidase (PPO). In addition, monoclonal antibody, TP80, to platelet glycoprotein II b-III a reacted with in about 26% of the blast cells. Chromosomal analysis of the peripheral blood revealed a mosaic pattern of a normal karyotype and abnormal ones, including 44, XY, -5, -7, -18, 10q-, +marker.     

A case of glycogen storage myopathy with acute heart failure

A case of 25-year-old woman with glycogen storage myopathy is reported here. She was hospitalized for acute heart failure after alcohol drinking. The electrocardiogram on admission showed marked ST elevation. Laboratory data showed elevated levels of serum myogenic enzymes but no rise in cardiomyogenic enzyme: CK 3862 IU/l CK-MB 35 IU/l, LDH 427 IU/l, GOT 203 IU/l. After several days, she recovered from acute heart failure and could walk without supporting. ST elevation in ECG and elevated myogenic enzymes were also normalized. The occurrence of acute myocardial infarction was ruled out because a coronary angiogram and 99 Tcm scintigram were normal. Physical examination revealed proximal muscular weakness and mental retardation (WAIS, total 72). Venous lactate response was normal after semi-ischemic forearm exercise. PAS staining of muscle specimen showed an excess deposit of glycogen. Ragged-red fibers were not seen on Gomori-trichrome stain. By electron microscopy, a large amount of glycogen particles were demonstrated in the subsarcolemma, but there were no abnormal mitochondrial changes. Biochemical analysis showed accumulation of glycogen in muscles: 28.7 mg/g muscle (normal 11.4 +/- 4.2 mg/g muscle). The activities of enzyme in the pathway of glycogen and glycogenosis (alpha-glucosidase, amylo-1,6-glucosidase, phosphorylase a, phosphorylase kinase, phosphofructokinase, etc.) were within normal limits. The spectrum of glycogen iodine complex was normal. Our case was different from any type of muscle glycogen storage disease previously reported. The etiology of an excess of glycogen deposit in muscles is unknown.     

Assessment of bilirubin clearance capacity of a newly developed ion-exchange adsorption column and its possible use as a supportive therapy in hepatorenal syndrome

We assessed the bilirubin reduction capacity of three different types of devices in vitro: a high-permeable membrane column for double-filtration plasmapheresis (DFP) (Evaflux 2A, Kuraray, Japan), and non-coated charcoal column for hemoperfusion (HP) (N-180, Asahi Medical, Japan), and ion-exchange columns for plasma adsorption (PA) (BR-350, Asahi Medical, Japan, and B-001, Kuraray, Japan). A column for DFP reduced the concentration of low-molecular proteins effectively such as plasma bilirubin and bile acids in an albumin-dependent manner. A charcoal column adsorbed low-molecular substances preferentially. But in these two columns, the loss of fibrinogen is a limiting factor for determining the processing plasma volume. Ion-exchange columns for PA adsorbed bile acids, disconjugated bilirubin, and monoconjugated bilirubin more efficiently compared with delta-bilirubin and unconjugated bilirubin. Pretreatment of the column with heparin reduced the loss of fibrinogen to less than 10%. We applied the BR-350 ion-exchange column in vivo for treatment of three patients with hyperbilirubinemia. After treatment, an alcoholic hepatitis patient with the hepatorenal syndrome (HRS) recovered from acute renal failure. However, in a patient with primary biliary cirrhosis and in a patient with fulminant hepatitis, the decrease of serum bilirubin was transient and no obvious beneficial responses were noted. The capacity and ability of the BR-350 column to adsorb plasma bilirubin was shown sufficient to treat deeply jaundiced patients, because 4 liters of the plasma of a patient with 108 mg/dl of initial total bilirubin concentration was able to be processed continuously without an obvious decrease in bilirubin adsorption capacity.(ABSTRACT TRUNCATED AT 250 WORDS)     

Successful treatment of a case of hepatocellular carcinoma with tumor necrosis factor and local hyperthermia.

A case of unresectable hepatocellular carcinoma which responded favorably to combined therapy with tumor necrosis factor (TNF) and local hyperthermia is reported. A 58-year-old man was admitted to our hospital in June 1988 for treatment of hepatocellular carcinoma affecting S4 and S8. After three sessions of transcatheter arterial embolization (TAE) therapy, the serum alpha 1-fetoprotein level decreased, and a reduction in the size of the lesions was also noted. Thereafter, the patient received local hyperthermia once a week (60 minutes of irradiation from a Thermotron-RF8 at 1,100W), but the alpha 1-fetoprotein level increased again in February 1989. On examination, enlargement of the S8 lesion and a new nodule in S7 were recognized. Since TAE was contraindicated due to liver dysfunction, human recombinant TNF (1 x 10(6)U) was given by intravenous infusion together with local hyperthermia once a week. Eight sessions of the combined therapy reduced the serum alpha 1-fetoprotein level markedly (7,512.0 to 2,782.0 pg/ml) and after eighteen sessions, 58.1% regression of tumor size (partial response) on computed tomography scans was observed. This anecdotal case supports previous experimental evidence suggesting that TNF plus hyperthermia may be effective for treating unresectable hepatocellular carcinoma.     

Solitary late recurrence of renal cell carcinoma

Solitary late recurrence is an unpredictable behavior pattern of renal cell carcinoma. We describe a patient with recurrence at the cranial bone 10 years after surgical management and another with recurrence at the sacral bone 13 years after treatment with radiotherapy and alpha-interferon. Both patients have been followed satisfactorily for 9 months. Unpredictable behavior of renal cell carcinoma makes lifelong followup of patients necessary. If a solitary recurrence is detected operative management definitely should be considered depending on the site of recurrence.     

Influence of ischemic heart disease on early and late mortality after aortic reconstruction for abdominal aortic aneurysm and aortoiliac occlusive disease

A retrospective study was undertaken to assess the influence of ischemic heart disease (IHD) on the early and late mortality of patients undergoing elective repair of an abdominal aortic aneurysms (AAA) or aortoiliac occlusive disease (AIOD). The patients were divided into IHD and non-IHD groups on routine clinical grounds. Among 157 patients with AAA, postoperative myocardial infarction occurred in 12.8% of the IHD group compared with 0.9% of the non-IHD group (P less than 0.05). The late mortality rate in the IHD group was higher than in the non-IHD group (p less than 0.05), and the mortality rate from myocardial infarction was 30% in the IHD group compared with 13% in the non-IHD group. Among 119 patients with AIOD, clinical evidence of IHD was found in 24 patients and extra-anatomical bypass was performed in 54% of these patients, compared with 35% of the patients in the non-IHD group. There was no occurrence of postoperative myocardial infarction. This study shows that an aggressive diagnostic approach should be taken for patients with AAA who have clinical evidence of IHD and that reevaluation of IHD should be performed in patients with AIOD after aortic reconstructive surgery.     

Effect of excisional wounding on DMBA -induced hamster tongue carcinogenesis

The purpose of this study was to investigate histologically the effect of wounding on the hamster tongue after pretreatment with DMBA. The animals in which the tips of the tongues were pretreated with DMBA for 8 weeks, subsequently excised, and had no treatment or received applications of acetone, showed epithelial dysplasias. The animals which had the same pretreatment, excision, and received additional post-excision applications of DMBA for 9–13 days, developed squamous cell carcinomas. However, the animals which were pretreated with DMBA for 8 weeks but had no excision, did not show any pathologic changes, even though they received additional applications of DMBA for 9–13 days. The animals which received no pretreatment with DMBA for 8 weeks hut had an excision, showed normal wound healing, even though they had a post-treatment with DMBA for 9-13 days. The results of the present study indicated that excisional wounding acted as a promotional stimulus in inducing the appearance of epithelial dysplasias or carcinomas of tongues initiated with the carcinogen DMBA.     

Studies on absorption, distribution and excretion of 14C labeled (+-)-7-(3-amino-1-pyrrolidinyl)-6-fluoro-1-(2,4-difluorophenyl)-1,4- dihydro-4-oxo-1,8-naphthyridine-3-carboxylic acid p-toluene-sulfonate hydrate (14C-T-3262) in rats and mice

Absorption, distribution and excretion of T-3262 were studied in rats and mice after oral administration of 14C-T-3262. The obtained results are summarized as follows. 1. 14C-T-3262 was absorbed from the upper small intestine such as duodenum in rats. 2. Serum levels of radioactivity in rats reached the highest concentration at 1 hour after an oral administration, then gradually diminished. 3. Urinary excretion was 35% and 42% of the dosed radioactivity in rats and mice, respectively, and fecal excretion was about 65% and 56% of the dosed radioactivity in rats and mice, respectively. 4. Biliary excretion in rats was about 27% of the dosed radioactivity after an oral administration of 14C-T-3262, and a half amount of excreted radioactivity was reabsorbed from the intestine. 5. Radioactivity was distributed the most into the kidney and the liver among all organs other the stomach and the intestine. Radioactivity was widely distributed into other organs such as spleen, adrenal, pancreas, lung, heart and thymus. But the distribution of radioactivity into the brain was little. 6. The distribution of 14C-T-3262 was also studied with whole body autoradiography in normal male mice and pregnant mice. The radioactivity was distributed widely to whole tissues except brain, spinal cord and eye ball. In pregnant mice, radioactivity levels in the fetuses were the same as the blood level of the mother mice. 7. The binding rate of 14C-T-3262 to rats and mice serum proteins was 63-66%. 8. Urinary and fecal excretion patterns of radioactivity in mice after multiple oral administration of 14C-T-3262 for 10 days were similar to those after a single administration. This result suggests that T-3262 did not accumulate in body. 9. After oral administration of 14C-T-3262 to nursing rats, the secreted radioactivity level in the milk was higher than the blood level.     

Anabolic steroid-associated hypogonadism in male hemodialysis patients

Hypogonadism in male hemodialysis patients has been previously reported. However, its precise pathogenesis has not yet been clarified. Mepitiostane and nandrolone decanoate are anabolic steroids prescribed for uremic anemia, and those may possibly exacerbate uremic gonadal damage. We studied the influences of these steroids on male gonadal function. Seventy-six hemodialysis patients were selected and examined for levels of luteinizing hormone (LH), follicular stimulating hormone (FSH), total testosterone, and prolactin. Twenty-three patients who received anabolic steroids showed lower testosterone values (205.2 +/- 35.6 ng/dl) than did patients without these steroids (449.7 +/- 21.3 ng/dl). Gonadotropins and prolactin showed no significant differences between the patients with and without the steroids. The testosterone values of three patients with mepitiostane increased after they stopped taking steroids. One patient suffering from complete aspermia recovered (sperm count: 0/ml to 1300 x 10(4)/ml) after discontinuation of mepitiostane and administration of human chorionic gonadotropin (HCG). This clinical study suggests that some anabolic steroids play a role in uremic hypogonadism; thus mepitiostane or its analogues should be carefully prescribed for young male patients.