Successful Treatment of Adult T-cell Leukemia/Lymphoma with MACOP-B,M-FEPA and VEPP-B Combination Chemotherapy

A 45-year-old man was referred to our department in March of 1989. Physical examination showed erythroderma, palmo-plantar hyperkeratosis, generalized lymphadenopathy, hepatosplenomegaly, and leukemic manifestation. The lymphocyte count in the peripheral blood before treatment was 1.7 × 10⁴ cells/mm³. Atypical lymphocytes such as flower cells and lobulated cells were seen in the peripheral blood. A sample excised from a lymph node showed immunoblastic, pleomorphic T cells by a modified classification scheme of the Working Formulation. A high level of serum LDH was detected (2.1 times the upper normal limit). Anti HTLV-1 antibody was also detected in the serum. The atypical lymphocytes were positive for CD3, CD4, CD5, CD7 and HLA-DR, and negative for CD8. Thus, the clinical, pathologic and immunologic features were those of typical acute-type ATL. The patient was treated with VEPA-M for three months starting in March of 1989. Because of poor response, the patient was then treated with MACOP-B, M-FEPA, and VEPP-B for about one year from June of 1989 and has been free of disease up to the time of writing, March of 1993.     

Frosted branch angiitis associated with Harada disease-like manifestations recurs 10 years later

PURPOSE: To describe a case with frosted branch angiitis occurring in association with manifestations of Harada disease. CASE: A 41-year-old man developed marked iridocyclitis, frosted branch angiitis and retinal white dots, together with serous detachment of the sensory retina in the posterior pole of both eyes. OBSERVATIONS: Fluorescein angiography demonstrated multiple retinal pigment epithelial leakage points in the early phase followed by late dye pooling in the serous detachment, consistent with the manifestations of Harada disease. The patient experienced recurrence of the same combination of manifestations 10 years later. On both occasions, he showed mononuclear cell increase and protein elevation in the cerebrospinal fluid on spinal tap. CONCLUSION: Fluorescein angiography and spinal tap supported the diagnosis of Harada disease in the patient. The present patient could be diagnosed as having either Harada disease with frosted branch-like response or frosted branch angiitis with serous retinal detachment as occurs in Harada disease.     

Breath-holding spells in somatoform disorder

Breath-holding spells (BHS) are commonly seen in childhood. However, there are no case reports of BHS occurring in adolescents or young adults. We report two young adult cases and discuss the pathogensis, both physically and psychologically. BHS occurred for 1-2 minutes after hyperventilation accompanied by cyanosis in both cases. Oxygen saturation was markedly decreased. Each patient had shown distress and a regressed state psychologically. These cyanotic BHS occurred after hyperventilation, and we considered that a complex interplay of hyperventilation followed by expiratory apnea increased intrathoracic pressure and respiratory spasm. Breath-holding spells can occur beyond childhood.     

Glycerol enhances CDDP-induced growth inhibition of thyroid anaplastic carcinoma tumor carrying mutated p53 gene

To increase the chemo-sensitivity of anaplastic thyroid carcinoma, we examined the effects of glycerol on the tumor growth after CDDP treatment. The cultured cells of an anaplastic thyroid carcinoma cell line (8305c) carrying a mutated p53 gene (mp53) were transplanted into the thighs of nude mice. Tumor growth was evaluated until 24 days after intraperitoneal injection of CDDP and/or pre-injection of glycerol to the tumor. We treated the mice with half the tumor volume of glycerol (1.2 M) and/or CDDP at 6 mg/kg (BW) either of which hardly inhibited tumor growth by itself. When we treated the mice with the combination of glycerol and CDDP at these concentrations, however, a clear delay of the tumor growth was observed. We also immunohistochemically analyzed the effects of glycerol on the induction of caspase-3 activity and apoptosis. Cells positive for cleavage to active caspase-3 and 85 kDa PARP, and apoptosis were hardly observed in the tumors when they were treated with glycerol or CDDP alone. In contrast, when they were treated with CDDP combined with glycerol, such positive cells were significantly increased. It has been shown that glycerol synergistically enhanced the effects of CDDP as a tumor suppressive agent through the induction of caspase-3-mediated apoptosis in 8305c tumors. Therefore, glycerol might be useful for chemotherapy in patients with mp53 cancer cells.     

Correction to: Swallowing evaluation by the Kuchikara Taberu Balance Chart and videoendscopic examination reveals that respiratory conditions,chewing, and position are strongly related to dysphagia

This study focused on the Kuchikara Taberu Balance Chart (KTBC) as a tool for swallowing function evaluation. To clarify the relationship between videoendoscopic (VE) examination of swallowing function and the KTBC, we compared median KTBC scores with and without laryngeal penetration identified by VE. Sixty-five patients with a mean age of 84.3 ± 7.9 years were examined at the Towada City Hospital. The patients were classified into groups based on laryngeal penetration, including 28 patients with and 37 patients without penetration. We found no significant differences in patient backgrounds. The median KTBC score (interquartile range) was 36.5 (31–44.5) in the group with laryngeal penetration and 42 (35–48.5) in the group without penetration, but the scores were not significantly different (level of statistical significance at α = 0.0036 determined by the Bonferroni correction method) when compared with the Mann–Whitney U test (36.5 vs. 42, z = -2.33, p = 0.020). The median respiratory condition (3 vs. 4, z = − 3.23; p < 0.0036), oral preparatory and propulsive phases (3 vs. 4, z = − 2.96; p < 0.0036), and position and endurance (1 vs. 3, z = − 3.25; p < 0.0036) scores were significantly lower in the group with laryngeal penetration. This study revealed a correlation between laryngeal penetration confirmed by VE and KTBC scores. Consequently, respiratory condition, oral preparatory and propulsive phases, and position and endurance may be useful as tools for the assessment of swallowing. In particular, we recommend adding respiratory status to dysphagia screening.

     

Hepatic CT perfusion measurements: A feasibility study for radiation dose reduction using new image reconstruction method

Objectives

To assess the effects of image reconstruction method on hepatic CT perfusion (CTP) values using two CT protocols with different radiation doses.

Materials and methods

Sixty patients underwent hepatic CTP and were randomly divided into two groups. Tube currents of 210 or 250 mA were used for the standard dose group and 120 or 140 mA for the low dose group. The higher currents were selected for large patients. Demographic features of the groups were compared. CT images were reconstructed by using filtered back projection (FBP), image filter (quantum de-noising, QDS), and adaptive iterative dose reduction (AIDR). Hepatic arterial and portal perfusion (HAP and HPP, ml/min/100 ml) and arterial perfusion fraction (APF, %) were calculated using the dual-input maximum slope method. ROIs were placed on each hepatic segment. Perfusion and Hounsfield unit (HU) values, and image noises (standard deviations of HU value, SD) were measured and compared between the groups and among the methods.

Results

There were no significant differences in the demographic features of the groups, nor were there any significant differences in mean perfusion and HU values for either the groups or the image reconstruction methods. Mean SDs of each of the image reconstruction methods were significantly lower (p < 0.0001) for the standard dose group than the low dose group, while mean SDs for AIDR were significantly lower than those for FBP for both groups (p = 0.0006 and 0.013). Radiation dose reductions were approximately 45%.

Conclusions

Image reconstruction method did not affect hepatic perfusion values calculated by dual-input maximum slope method with or without radiation dose reductions. AIDR significantly reduced images noises.     

Case of Gerstmann-Str?ussler-Scheinker syndrome (GSS-P102L) mimicking variant Creurtzfeldt-Jakob disease in clinical manifestation and MRI findings]

We reported a 51-year-old woman with Gerstmann-Str?ussler-Scheinker syndrome (GSS P102L) manifesting characteristic MRI findings. At the age of 45, She developed gait disturbance with muscle atrophy in the lower limbs and positive plantar flexor sign. Subsequently, sensory disturbance such as refractory pain in the lower limbs and ataxic gait were developed at the age of 49. Following these clinical symptoms, she finally demonstrated rapid progressive cognitive dysfunction. Just after presenting cognitive dysfunction, cranial MRI was performed. Cranial MRI with diffusion-weighted imaging and FLAIR imaging demonstrated abnormal high intensity lesions in the bilateral pulvinar, caudate nuclei and cerebral cortex. The degree of high signal at the pulvinar was less than those of the cortex and caudate nuclei. A proline-for-leucine substitution at codon 102 of the prion protein gene was demonstrated. These results allowed the diagnosis of GSS (P102L). This is a rare case of GSS (P102L) presenting with high intensity lesions in the bilateral pulvinar on MRI.     

Assessing Proteinase K Resistance of Fish Prion Proteins in a Scrapie-Infected Mouse Neuroblastoma Cell Line

The key event in prion pathogenesis is the structural conversion of the normal cellular protein, PrP^C, into an aberrant and partially proteinase K resistant isoform, PrP^Sc. Since the minimum requirement for a prion disease phenotype is the expression of endogenous PrP in the host, species carrying orthologue prion genes, such as fish, could in theory support prion pathogenesis. Our previous work has demonstrated the development of abnormal protein deposition in sea bream brain, following oral challenge of the fish with natural prion infectious material. In this study, we used a prion-infected mouse neuroblastoma cell line for the expression of three different mature fish PrP proteins and the evaluation of the resistance of the exogenously expressed proteins to proteinase K treatment (PK), as an indicator of a possible prion conversion. No evidence of resistance to PK was detected for any of the studied recombinant proteins. Although not indicative of an absolute inability of the fish PrPs to structurally convert to pathogenic isoforms, the absence of PK-resistance may be due to supramolecular and conformational differences between the mammalian and piscine PrPs.