PubMed Health⌕ Search

Biomedical subjects

Andrea Ponyi

Publications and source records attributed to Andrea Ponyi.

12 recordsLinked to original sources
PubMed2006

[Hungarian experience with non-Hodgkin's lymphoma in childhood].

Between 1990 and 2004, 230 children with non-Hodgkin's lymphoma (NHL) were treated according to the Berlin-Frankfurt-Münster (BFM) protocols (NHL-BFM-90 and -95) in Hungary. The aim of the present study was to summarize our experience with these protocols, to assess the survival rates and to compare the Hungarian data with the international results. The male-to-female ratio was 2.59:1, the mean age at the time of diagnosis was 10 years and 1 month. Ninety-one children had lymphoblastic/T-NHL (LB/T-NHL), 108 B-NHL and 31 anaplastic large cell lymphoma (ALCL). Twenty-eight patients had relapse after a mean time of 13 months from the time of the initial diagnosis. In the above mentioned period, 16 children underwent autologous stem-cell transplantation. Nine patients with B-NHL got anti-CD20 immunotherapy. The five-year overall survival (OS) of our patients is 77.8%+/-3%, the event-free survival (EFS) is 75.1%+/-3%. The 5-year OS and EFS rates were not statistically different in the three histology groups (OS: 71.6%+/-5%, 82.7%+/-4% and 80.3%+/-7%; EFS: 68.7%+/-5%, 81.1%+/-4% and 73.9%+/-8% in LB/T-NHL, B-NHL and ALCL, respectively). We can conclude that non-Hodgkin's lymphoma has a quite good prognosis among the malignant pediatric diseases. The cure rate is over 75%. The Hungarian results are comparable with other international data. In the last five years the mortality during induction was reduced from 10% to 2% and the OS is about 10% better than it was before. In case of relapse or residual disease, therapeutic results can be improved with stem-cell transplantation with or without immunotherapy.

Adolescent↗
PubMed2005

Prostate cancer underlying acute, definitive dermatomyositis: successful treatment with radical perineal prostatectomy.

The idiopathic inflammatory myopathies are systemic autoimmune diseases characterized by chronic inflammation leading to progressive weakness of the proximal muscles. In 7-66% of cases of adult dermatomyositis different malignant tumours can promote the difficult cascade mechanisms at the cell level, leading to rapid weakness of skeletal muscles [1]. We report on a patient with all characteristic signs of acute, severe dermatomyositis associated with a low-grade, low-stage prostate cancer cured by radical perineal prostatectomy.

Adenocarcinoma↗
PubMed2005

Paraneoplastic rheumatic syndromes.

Paraneoplastic symptoms caused by a malignancy but not directly related to tumour invasion are the result of a wide variety of tumour-derived biologic mediators, such as hormones, peptides, antibodies, cytotoxic lymphocytes, autocrine and paracrine mediators. Recognition of paraneoplastic syndromes is important, as it may lead to an early diagnosis of cancer. On the other hand, the clinical severity of the symptoms can be used as a guide to the extent of response to underlying tumour therapy. The quality of life of the patient is affected, therefore the palliative treatment of paraneoplasia is very important.

Humans↗
PubMed2005

Detection of TT virus in patients with idiopathic inflammatory myopathies.

The TT virus, a recently identified single-stranded DNA virus with unknown pathogenicity, has been shown to commonly infect humans. Viruses have been considered to contribute to disease pathogenesis in autoimmune disorders including idiopathic inflammatory myopathies (IIMs) and rheumatoid arthritis (RA). We assessed the prevalence of TTV infection in IIM compared with that in patients with RA and healthy blood donors. Detection of TTV was conducted by nested PCR and real-time PCR in the sera of 94 patients with IIM, 95 RA patients. and 95 age- and sex-matched healthy blood donors. Identity of the PCR products was confirmed by sequencing. TTV DNA was detected in 61 of 94 (64.9%) patients with IIM, in 64 of 95 (67.4%) patients with RA, and in 62 of 95 (65.3%; P > 0.05) healthy individuals. Age, sex, activity, or duration of disease had no influence on TTV positivity in either group. However, patients with severe IIM (n = 36) had a significantly higher rate of TTV infection (31/36, 86.1%) than patients with mild disease (30/58, 51.7%, P < 0.05, chi(2) = 10.0). Disease was considered severe in IIM when immunosuppressive treatment was necessary because of continuous high activity and/or serious inner-organ involvement despite corticosteroid treatment. In conclusion, although we found the detection rate of TTV similar in patients with idiopathic inflammatory myopathies and rheumatoid arthritis and comparable to that in healthy controls, our data suggest that infection with TT virus may result in a more severe disease in patients with idiopathic inflammatory myopathies.

Adult↗
PubMed2005

Cancer-associated myositis: clinical features and prognostic signs.

Idiopathic inflammatory myositis is characterized by progressive weakness of the proximal muscles. There is a higher risk of malignancy than in the normal population. The aim of this study was to evaluate the frequency of malignancy among 251 myositis patients. We also compared clinical and immunological characteristics of cancer-associated myositis with primary myositis. There were no malignancies among polymyositis, overlap, or juvenile myositis patients. Twenty-two of ninety dermatomyositis patients also had a malignant disease. Patients with cancer-associated dermatomyositis were significantly older than primary myositis patients and had more severe cutaneous and muscle symptoms. Dysphagia and diaphragmatic involvement were more frequent among cancer-associated patients, while extramuscular features were less frequent. After successful treatment of the malignancy, we were able to manage myositis symptoms. One-year survival rate was significantly better in primary dermatomyositis patients. The subset of cancer-associated myositis differs from primary myositis in many aspects of its clinical and immunological features. Prognosis and life expectancy in cancer-associated myositis patients is determined by the underlying malignant disease. Therefore, age- and sex-specific examinations for detection of an underlying malignancy are important in the management of patients with dermatomyositis.

Adult↗
PubMed2004

Antiphospholipid and antisynthetase syndrome in a patient with polymyositis-rheumatoid arthritis overlap.

Idiopathic inflammatory myopathies, mainly polymyositis (PM) may occur in the course of several autoimmune diseases. The overlapping forms of myositis, when the patient also meets the criteria for rheumatoid arthritis (RA), affect 3%-5% of myositis patients [1]. To the best of our knowledge this is the first report on the overlapping form of RA, antiphospholipid syndrome (APS) and the serological subgroup of PM, the antisynthetase syndrome (ASS).

Adult↗
PubMed2004

Long-term survival of patients with idiopathic inflammatory myopathies according to clinical features: a longitudinal study of 162 cases.

The idiopathic inflammatory myopathies are characterized by chronic muscle inflammation and involvement of internal organs, which contribute considerably to the morbidity and mortality of the disease. We conducted the current study to determine the survival data for patients with idiopathic inflammatory myopathies according to the presence of extramuscular clinical manifestations. We also determined the cumulative survival probability and the long-term prognosis and analyzed the causes of death at a single clinical immunology center.A survival analysis was performed using data for 162 patients diagnosed between 1976 and 1997 according to Bohan and Peter's criteria. Patients were followed up for a minimum of 5 years (median, 101.5 mo) or to date of death. Cumulative survival probability was calculated by the Kaplan-Meier method. The influence of extraskeletal and extramuscular involvement was analyzed as prognostic factors for death by Cox proportional hazards survival model. Eighteen disease-specific deaths occurred; pulmonary and cardiac complications were the most frequent causes of death. Global survival rates were 95%, 92%, and 89% for 1, 5, and 10 years, respectively. Analysis for clinicopathologic subgroups revealed that cancer-associated myositis had the worst prognosis, while juvenile and overlap myositis had the best prognosis. Five- and 10-year survival rates were 94.2% and 89.4% for patients with primary polymyositis and 90.1% and 86.4% for primary dermatomyositis patients, respectively. In the whole group of patients with idiopathic inflammatory myopathy, cardiac (p < 0.01) and respiratory muscle involvement (p = 0.045) were significant prognostic factors for death. In the group of patients with primary polymyositis/dermatomyositis, cardiac involvement was the main prognostic factor for death (p < 0.01). Myositis patients described in this study have higher survival rates than reported previously worldwide. We examine the reasons for the differences between the data in the current study and the available survival data in the relevant literature.

Adult↗
PubMed2004

Intracellular IL-4/IFN-gamma producing peripheral T lymphocyte subsets in B cell non-Hodgkin's lymphoma patients.

AIM: The availability of several biological therapy options is rapidly growing in the field of malignant lymphomas. This emphasizes the need to understand the precise interaction of the host immune system with the malignant disease. We measured the intracellular cytokine responses in lymphoma patients' lymphocytes, to characterize the polarization changes in their immune system. METHODS: Patients with B cell non-Hodgkin's lymphoma were involved in the study. Peripheral lymphocytes were labeled with anti-CD4 or anti-CD8 antibodies and intracellular accumulation of IL-4 or IFN-gamma was detected after in vitro incubation the cells with activating cocktail. The frequency of different T-cell subsets were measured within the lymphocyte population. RESULTS: Significantly increased CD4+, IFN-gamma producing (Th1) cell percentage were found in untreated lymphoma cases (28.8% vs. 21.8%). CD8+ IL-4 and IFN-gamma producing (Tc0) T cell frequency is significantly higher in untreated lymphoma patients compared with normal controls (1.3% vs. 0.47%). The frequency of CD4+ IL-4 producing (Th2) cells is significantly lower in untreated patients (0.96% vs. 1.19%). Patients in long-term remission have lower frequency of CD4+, IL4 producing (Th2) cell ratio (0.31% vs. 1.19%) and increased CD4+ IFN-gamma producing (Th1) cell frequency (30.1% vs. 21.8%), compared with healthy normal controls. CONCLUSION: Our results demonstrate that there is a sustained increase in the CD4+ IFN-gamma producing cell frequency in lymphoma patients. The frequency of CD4+ IL-4 producing cells is decreasing by treatment. These may contribute to strong polarization toward Th1 type response, needed for lymphoma clearance and remission.

Adult↗
PubMed2003

[Clinical characteristics of juvenile dermatomyositis].

INTRODUCTION: Dermatomyositis, belonging to the group of the idiopathic inflammatory myopathies, is characterized by bimodal pattern of age-specific incidence of rates, with peaks in age group from 5 to 14 years (juvenile dermatomyositis) and in age group from 45 to 64 years (adult dermatomyositis). THE AIM OF THIS STUDY: Is to evaluate the clinical characteristics of 12 patients with juvenile dermatomyositis followed by the 3rd Department of Internal Medicine, University of Debrecen and the 2nd Department of Pediatrics, Semmelweis University, Budapest. METHODS: The authors analyzed the medical records of the patients with juvenile and adult form of dermatomyositis retrospectively. RESULTS: All of the children had symmetrical weakness of the proximal muscles. The most frequent cutaneous features were facial erythema and Gottron papules (11/12). The extramuscular manifestations were also assessed. 7 children had arthralgia. There were observed pulmonary fibrosis, Raynaud-syndrome, dysphagia and sicca-syndrome in the same patient, whose disease is overlapped with progressive systemic sclerosis. In view of the clinical course, the authors found that prevalence of polycyclic (relapsing-remitting) and monophasic subtypes of the disease were similar (6/12 and 5/12). Finally, all of the patients achieved remission, however, 2 patients have to take low-dose corticosteroid therapy permanently to maintain remission. One patient's cutaneous symptoms proved to be persistent and in further 2 cases, regression of the calcinosis is slow, but continuous. DISCUSSION: The authors compare their data of juvenile patients with the data of the relevant literature and to their experience with the management of adult DM patients. It seems to be reasonable to treat the patients in centres.

Adolescent↗
PubMed2002

[Myositis associated with tumors].

AIMS OF THE STUDY: In the 214 polymyositis / dermatomyositis patients in our department we studied the association of polymyositis / dermatomyositis with malignant tumors, the clinical specificities and therapeutic response changes in these cases. METHODS: Retrospective analysis of the data available since 1985 of the patients treated at the DEOEC 3(rd) Department of Internal Medicine. RESULTS: From 60 patients with dermatomyositis 9 were diagnosed with neoplasia. In 5 patients simultaneously with the dermatomyositis diagnosis, in the other 4 patients 2-2-3-7 years after the onset of dermatomyositis. In the 154 patients with polymyositis we did not observe development of tumors. CONCLUSIONS: Neoplasm was observed in 15% of patients with dermatomyositis. The patients presented with serious skin involvement which did not respond well to the dermatomyositis treatment. The patients in whom tumors developed simultaneously with the dermatomyositis required more aggressive treatment. After the therapy of the tumor (surgery, radiotherapy), the skin and muscle symptoms responded better to the immunosuppressive therapy.

Aged↗