Blast Crisis of Chronic Myelogenous Leukemia with Tumor Formation Characterized by T-cell Features--A Case Report

We report a case of chronic myelogenous leukemia (CML) associatedwith pronounced peripheral lymphadenopathy, with the cells havingthe Philadelphia (Phl) chromosome and T-cell features. A 23-year-oldman who was diagnosed as having CML and treated with busulfanwas admitted to our hospital because of increasing hepatosplenomegalyand pronounced lymphadenopathy. An axillary lymph node biopsydisclosed that the malignant cells formed rosettes with neuraminidase-treatedsheep red blood cells (En) (95.0%) and were positive for Leu1 (91.8%). Of the cytochemical reactions, peroxidase was negativeand periodic acid-Shiff, acid -naphthyl acetate esterase andß-glucuronidase were all positive. The karyotype ofthe bone marrow cells was 46 XY Phl positive (22q–), andthat of the lymph node cells was 51 XY Phl positive +8, +9,+18, +19, +21, 22q–. He was treated with various anti-leukemicagents and irradiation. Despite such treatments, he died ofpneumonia. This is a report of a CML patients with blast crisisand tumor formation characterized by T-cell features.     

Characteristics of juvenile dermatomyositis in Japan

Questionnaires were sent to 1290 hospitals in Japan asking for data on patients with juvenile dermatomyositis (JDM) diagnosed between June 1984 and May 1994. Of the 204 patients identified by these questionnaires, 102 met the criteria for JDM. JDM is categorized into three subtypes: Banker-type JDM , Brunsting-type and fulminant-type; patients with the latter exhibit markedly elevated serum levels of creatinine phosphokinase (> 10 000 U/mL) and appear to be at risk of renal failure. Cutaneous manifestations were present in 98% of patients and preceded the appearance of other symptoms. This tendency is one of the reasons for the difficulty in some cases in diagnosing the onset of JDM. Better criteria for early treatment of JDM are needed. The results of the present study suggest that itching and calcinosis are factors that indicate a poor prognosis in patients with JDM. Muscle enzyme levels do not always reflect disease activity, suggesting that methods other than measurement of muscle enzymes, such as measurement of the levels of neoprerin and von Willebrand factor antigen, as well as magnetic resonance imaging should be used to be evaluate disease severity. Patients with Brunsting-type JDM who exhibit dysphagia and antinuclear antibody positivity and patients with Banker-type JDM should be treated aggressively. Pulse therapy should be selected as the initial therapy in patients with fulminant-type JDM.     

Aqueous fraction of Sauropus androgynus might be responsible for bronchiolitis obliterans

Background and objective: Bronchiolitis obliterans (BO) has been reported to develop following ingestion of Sauropus androgynus (SA), a leafy shrub distributed in Southeast Asia. Little is known about direct effects of SA on airway resident cells or haematopoietic cells in vitro. Identification of the SA component responsible for the development of BO would be an important key to elucidate its mechanism. We sought to elucidate the direct effects of SA on airway resident cells or haematopoietic cells and identify the SA element responsible for the pathogenesis of BO. Methods: SA dry powder was partitioned into fractions by solvent extraction. Human and murine monocytic cells, epithelial cells and endothelial cells were cultured with SA solution or fractions eluted from SA. We also investigated the effect of SA in vivo using a murine BO syndrome (BOS) model. Results: The aqueous fraction of SA induced significant increases of inflammatory cytokine and chemokine production from monocytic lineage cells. This fraction also induced significant apoptosis of endothelial cells and enhanced intraluminal obstructive fibrosis in allogeneic trachea allograft in the murine BOS model. We found individual differences in tumour necrosis factor α (TNF‐α) production from monocytes of healthy controls stimulated by this aqueous fraction of SA, whereas it induced high‐level TNF‐α production from monocytes of patients with SA‐induced BO. Conclusions: These results suggest that an aqueous fraction of SA may be responsible for the pathogenesis of BO.     

Transcapillary ultrafiltration and peritoneal equilibration test in pediatric patients

The usefulness of the peritoneal equilibration test (PET) in children is unknown. The relationship between transcapillary ultrafiltration and PET was investigated in order to evaluate the usefulness of PET in children. PET was performed on 14 patients undergoing peritoneal dialysis. Their age and bodyweight ranged from 3.8 to 23.6 years and 10.2 to 55.8 kg, respectively. The patients were divided into two groups according to bodyweight; group A patients weighed ≤ 40 kg (n = 7) and group B patients weighed > 40 kg (n = 7). There was no significant difference in the mean infusion volume per bodyweight between the two groups, but the mean infusion volume per body surface area was smaller in group A than in group B. Group A showed a more rapid equilibration of dialysate glucose and creatinine than group B. Higher normalized mass transfer area coefficients were evident in group A. In spite of the lower effective glucose gradient in group A, the transcapillary ultrafiltration capacity (TUFC) showed no difference between the two groups. When the slope indices of the regression equations between the two groups were compared, the slopes of the regression in the relationship between TUFC and dialysate (D) ratios D/D₀ glucose or D/P creatinine in group A were steeper than those in group B. Results of the present study indicate that the larger peritoneal area to infusion volume in patients with smaller body size results in both a rapid equilibration of solutes and sufficient transcapillary ultrafiltration.     

Personality differences in the Munich Personality Test between patients with major depression and panic disorder

This study attempted to determine whether patients with major depression and panic disorder could be differentiated by personality features, measured by the Munich Personality Test (MPT). One of the six MPT personality dimensions, `rigidity', was developed in relation to the `melancholic type of personality', which may be a specific personality feature of depressive subjects. We therefore hypothesized that the MPT might be sensitive to possible personality differences between patients with major depression and panic disorder. Sixty-six patients with major depression and 27 patients with panic disorder, taken from consecutive intakes at an outpatient unit, were compared in terms of six personality dimensions of the MPT. The results demonstrated that rigidity could significantly differentiate the two patient groups, even after the possible confounding effects on the personality assessments were statistically partialled out. The MPT was suggested to be powerful for describing distinctive personality features of depressive subjects from anxiety subjects.     

Alternating Non-Cross Resistant Chemotherapy for Small Cell Lung Cancer

After stratification for the extent of disease, previously untreatedpatients .with small cell lung cancer randomized to receivetherapy with the four-drug combination of cyclophosphamide,oncovin, nimustine hydrochloride (ACNU), and procarbazine (CONP)every four weeks (continuous regimen) or to receive CONP alternatingwith the three-drug combination of etoposide (VP-16), adriamycinand cisplatin (VAD) at four-week intervals (alternating regimen).Sixty-nine patients were entered in the study. Of 34 evaluablepatients receiving the continuous regimen, six (17.6%) achievedcomplete response (CR) and 16 (47.1%) achieved partial response(PR). Of 31 evaluable patients receiving the alternating regimen,10 (32.3%) achieved CR, and 16 (51.6%) achieved PR. There wasa tendency in favor of the alternating regimen in CR and overall response rates (0.05 < p < 0.1). There were no significantdifferences be tween the regimens in response duration or survival.The projected median survival times were 9.2 months and 9.4months for the continuous and alternating regimens, respectively.One patient receiving the continuous regimen and three receivingthe alternating regimen have been living for more than two years.The major toxicity was myelosuppression in both regimens. Onepatient died of hemorrhage due to thrombocytopenia during inductionwith CONP, and one patient died of cisplatin-induced renal failure.We conclude that alternating non-cross resistant chemotherapyleads to improved CR and response rates, but does not improvesurvival.     

Electrophysiologic Evaluation of Asymptomatic Patients with the Wolff-Parkinson-White Pattern

In the past 4 years, 34 asymptomatic patients with the Wolff-Parkinson-White (WPW) pattern underwent electrophysiologic study. The effective refractory period (ERP) of antegrade conduction over the accessory pathway was 288 +/- 29 msec. In three asymptomatic patients (9%), the antegrade ERP of the accessory pathway was shorter than 250 msec. The antegrade ERP of the accessory pathway became shorter than 250 msec in an additional 12 of 22 (55%) patients after isoproterenol administration. Nineteen (56%) of the asymptomatic patients showed the absence of retrograde conduction over the accessory pathway even after isoproterenol administration. The rate of induction of orthodromic reciprocating tachycardia in the asymptomatic WPW patients was 15% (5/34), which was significantly lower than that in the symptomatic patients. These data suggest that in the asymptomatic patients, the absence of retrograde conduction over the accessory pathway is the reason they remained asymptomatic, free of reciprocating tachycardia. However, even in the asymptomatic patients, some had the accessory pathway in which antegrade ERP was shorter than 250 msec. They may result in rapid ventricular conduction over the accessory pathway when atrial fibrillation develops.     

New Electrophysiologic Features and Catheter Ablation of Atrioventricular and Atriofascicular Accessory Pathways:

Catheter Ablation for Mahaim Pathways. Introduction : Several modalities of catheter ablation have been proposed to eliminate Mahaim pathway conduction. However, limited research has been reported on the electrophysiologic nature of this pathway in its entity.
Methods and Results : In seven patients, electrophysiologic study was performed, and radiofrequency energy was applied to investigate the electrophysiologic clues for successful ablation. In all seven patients, the Mahaim pathway was diagnosed as a right-sided atriofascicular or atrioventricular pathway with decremental properties. In two patients, two different kinds of electrograms were recorded through the ablation catheter positioned at the Mahaim pathway location: one was suggestive of conduction over the decremental portion, demonstrating a dulled potential; and the other of nondecremental conduction, demonstrating a spiked potential. All but one of the Mahaim pathways were eliminated successfully at the atrial origin where the spiked Mahaim potential was recorded. Radiofrequency energy application was performed at the slow potential site resulting in failure to eliminate the conduction over the Mahaim pathway. Conduction block at the site between the slow and fast potential recording sites was provoked by intravenous administration of adenosine, concomitant with a decrease in the amplitude of the Mahaim potential. In one patient, the clinical arrhythmia was a sustained monomorphic ventricular tachycardia originating from the ventricular end of the Mahaim fiber.
Conclusion : The identification of Mahaim spiked potentials may be the optimal method to permit their successful ablation. Detailed electrophysiologic assessment is indispensable for successful ablation of tachycardias associated with Mahaim fibers because tachycardias unassociated with Mahaim fibers can occur despite complete elimination of the Mahaim fiber.     

Suppression of Torsades de Pointes by Biventricular Pacing in a Patient with Long QT Syndrome

This report describes a case of a patient with long QT syndrome (LQTS) with recurrent episodes of torsades de pointes (TdP). Use of biventricular pacing (BiVP) resulted in a shorter QT interval and a shorter T‐peak‐end interval and prevented further episodes of TdP. These findings suggest that BiVP may be helpful in patients with LQTS and refractory TdP.     

Intracoronary Infusion of Dilute Ethanol for Control of Ventricular Rate in Patients with Atrial Fibrillation

The effects of selective infusion of 25% ethanoi into the AV nodal artery was assessed in 1J putients with atriai fibrillation and uncontrollabiy rapid ventricular response rates. The primary study objective was to achieve permaneni modificafion of AV nodal function and control ventricular rate without drug therapy and mthout causing permanent complete AV block. "Clinical success" was defined as drug-free rate control by either AV nodal modification or the production of complete AV block. Selective catheterization and ethanol infusion into the AV nodal artery could be performed in nine patients. Jntracoronary ethanol infusion acutely caused second- or third-degree AV noda] block in seven palients and an increase in AV nodal refractory period and Wenckebach cycle length in two patients. Acute occlusion of the AV nodal artery or infarction of nontarget myocardium was not observed. During follow-up of 22.2 ± 2.2 months the primary study objective was attained in only four of nine patienfs treated, yielding an efficacy of 44%. However, the "clinical success" rate was 78%. The acute effects of ethanol on AV conduclion did not predict the chronic effects. Selective intracoronary infusion of dilute ethanol lo control the ventricular rate in atrial fibrillation should be considered when rodiofrequency ablation has been unsuccessful. This method of chemical ablation is as effective and probably safer than rapid administration of 96% ethanol.