Induction therapy by frequent administration of doxorubicin with four other drugs, followed by intensive consolidation and maintenance therapy for adult acute lymphoblastic leukemia: the JALSG-ALL93 study.

In order to improve the disappointing prognosis of adult patients with acute lymphoblastic leukemia (ALL), we applied similar induction therapy as that used for acute myeloid leukemia (AML), ie frequent administration of doxorubicin (DOX). DOX 30 mg/m(2) was administered from days 1 to 3 and from days 8 to 10 together with vincristine, prednisolone, cyclophosphamide and L-asparaginase, followed by three courses of consolidation and four courses of intensification. From December 1993 to February 1997, 285 untreated adult patients with de novo ALL were entered. Of 263 evaluable patients (age 15 to 59; median 31), 205 (78%) obtained complete remission (CR). At a median follow-up period of 63 months, the predicted 6-year overall survival (OS) rate of all patients was 33%, and disease-free survival (DFS) rate of CR patients was 30%, respectively. By multivariate analysis, favorable prognostic factors for the achievement of CR were age <40 and WBC <50 000/microl; for longer OS were age <30 and WBC <30 000/microl; and for longer DFS of CR patients were FAB L1 and ALT <50 IU/l. Among 229 patients who had adequate cytogenetic data, 51 (22%) had Philadelphia (Ph) chromosome. Ph-negative chromosome was a common favorable prognostic factor for CR, longer OS and DFS. DFS was not different between early sequential intensification (n = 48) and intermittent intensification (n = 43) during the maintenance phase. Among CR patients under 40 years old, the 6-year survival was not different between the allocated related allo-BMT group (34 patients) and the allocated chemotherapy group (108 patients). However, among patients with Ph-positive ALL, the survival of patients who actually received allo-BMT was superior to that of patients who received chemotherapy (P = 0.046).     

Conflict of intentions due to callosal disconnection

OBJECTIVES: Three patients with callosal syndrome manifested a peculiar symptom in that they were unable to perform intended whole body actions because another intention emerged in competition with the original one. Attempts were made to clarify the symptomatology of this manifestation and its possible mechanism is discussed. METHODS: The three patients are described and previous reports on patients with callosal damage were reviewed. Four additional patients with similar symptoms were found and the clinical features common to all seven patients were examined. RESULTS: This symptom could not be attributed to unilateral movement disorders such as unilateral apraxia, intermanual conflict, or compulsive manipulation of tools. The manifestations included marked hesitation in initiating actions, interruption of actions, repetitive actions, and performance of unintended actions with difficulty in correcting them. All patients, except one, had a lesion in the posterior half of the body of the corpus callosum, and there was no significant involvement of the cerebral cortex. The symptom became manifest later than 4 weeks after callosal damage. It occurred during spontaneous actions, but not during well automated actions nor when following instructions. CONCLUSION: This symptom, tentatively named "conflict of intentions", can be regarded as a fragment of diagonistic dyspraxia originally described by Akelaitis, although it can occur independently of intermanual conflict. Normally, the right and left cerebral hemispheres may be complementarily modifying automated whole body actions in order to adapt behaviour to changes of the environment as well as to the intention. Partial callosal disconnection without significant cortical involvement would exaggerate the disparity between the role of each hemisphere through the reorganisation of neural systems after callosal damage. Such double, often contrary, behavioural tendencies may sometimes simultaneously enter the patient's awareness.     

Congenital muscular dystrophy with primary partial laminin alpha2 chain deficiency: molecular study

The authors report a case of congenital muscular dystrophy with mild nonprogressive muscle weakness, white matter hypodensity, and absence of the laminin alpha2 chain in muscle fibers with two antibodies, but not with four others. They identified mutations in LAMA2, which explain the partial laminin alpha2 deficiency. Analysis of this case and two others allows us to refine the epitopes of two of the commercial antibodies, and illustrate the importance of using antibodies directed against different domains of the protein.     

P-Glycoprotein Is Positively Correlated with p53 Protein Accumulation in Human Colorectal Cancers

To explore the relationship between mutant p53 and Pgp expression, we have examined the levels of both proteins in human colorectal adenocarcinomas. Serial frozen sections of 40 surgical samples were stained with an anti-Pgp (MRK16) and two different anti-p53 protein antibodies (Abs), PAb421 and Pabl80l. Nineteen (47.5%) of 40 samples examined were positive for Pgp, and 18 (45%) of 40 were positive for p53. The samples that stained positively with PAb421 also stained positively with Pahl80l. Pgp expression was detected in 13 (76.5%) of 17 samples that were positive for p53 using PAb421 and in 15 (83.3%) of 18 samples that were positive for p53 using Pabl80. Thus, we found that p53 and Pgp were co-expressed in a significant number of samples ( P < 0.002). There was no relationship between Pgp or p53 protein accumulation and histologic grade or stage. The present results demonstrate that Pgp expression is closely associated with p53 protein accumulation in human colorectal cancers. These data provide evidence to support the idea that mutant p53 activates the MDR1 gene in vivo .     

Isolation of adenovirus type 11 from the brain of a neonate with pneumonia and encephalitis

We describe a fatal case of adenovirus pneumonia accompanied by encephalitis in a neonate who showed lethargy on the 6th day and died on the 12th day. Adenoviral particles as well as viral intranuclear inclusions were noted in pulmonary alveolar epithelium cells. Neuropathological examination revealed diffuse oedema, perivascular cuffing and gliosis in the white matter. Adenovirus type 11 was isolated from lung, hilar lymph node, and brain tissue. This is the first instance of adenovirus isolation from brain tissue in a newborn infant. The viro-logical and neuropathological findings sugest the invasion of neural tissue by adenovirus and substantiate the significance of neurological symptoms observed in neonatal adenovirus infection.     

5-day vs. 7-day triple therapy with rabeprazole, clarithromycin and amoxicillin for Helicobacter pylori eradication

AIM: To determine whether a 5-day regimen with rabeprazole, clarithromycin and amoxicillin (RCA) was as effective as a 7-day regimen. METHODS: A total of 139 H. pylori-infected patients were randomized to receive either a 5-day or 7-day course of rabeprazole 10 mg b.d., clarithromycin 400 mg b.d. and amoxicillin 750 mg b.d. Eradication was assessed by CLO test, histology and 13C-urea breath test. RESULTS: On the intention-to-treat basis, eradication rates were 66% (46 out of 70) and 84% (58 out of 69) for the 5- and 7-day regimens, respectively (P < 0.05). Using per protocol analysis, eradication rates were 70% (46 out of 66) and 91% (58 out of 64) for the 5- and 7-day regimens, respectively (P < 0.01). Adverse events, which were observed in 14 patients from each group, caused discontinuation of treatment in only two patients, resulting in excellent compliance. CONCLUSIONS: Our 5-day regimen of RCA yielded inferior results, whereas the 7-day regimen achieved an eradication rate exceeding 90% on the per protocol basis. Therefore, treatment regimens of less than 7 days for proton pump inhibitor-clarithromycin-amoxicillin therapies cannot be recommended.     

A case of non-metastatic giant mucinous carcinoma of the breast

A surgically resected case of giant mucinous carcinoma of the breast that had remained untreated for 2 years is reported. A 64-year-old postmenopausal woman presented with a large right breast mass (17.4 x 16.5 x 14.5 cm). Although she had noticed a mass in the right breast 2 years previously, she had not sought treatment. Mucinous carcinoma was diagnosed by core needle biopsy and she underwent right modified radical mastectomy with a free skin graft. There were no lymph node metastases or distant metastases. Fourteen months postoperatively, she remains well without evidence of tumor recurrence. Although several reports have suggested that pure mucinous carcinoma of the breast has a favorable prognosis, we need to follow this case until the clinical behavior and the outcome become clear.