Lege Artis Medicinae

[LATE COMPLICATION OF EXTRANODAL LYMPHOMA FOLLOWING DERMATOMYOSITIS]

VÁNCSA Andrea, PONYI Andrea, CONSTANTIN Tamás, GERGELY Lajos, DANKÓ Katalin

JULY 20, 2004

Lege Artis Medicinae - 2004;14(07)

[INTRODUCTION - Intramuscular follicular Bcell lymphoma is a rare entity of malignant lymphomas complicating the disease course of dermato/polymyositis. CASE REPORT - The authors report a female patient who was diagnosed with dermatomyositis in 1963 (age of 36). She was given steroid and hydroxychloroquine therapy. In 1973 she was treated with steroid for Boeck sarcoidosis. In 1999, she was treated for steroid therapy again for reoccurence of dermatomyositis. In 2000, a histological diagnosis of primary intramuscular follicular lymphoma was established from the right thigh region. She was given several courses of polychemotherapy and radiation therapy, but the lymphoma was difficult to control, and she died of progressive disease in 2003. CONCLUSION - It is an interesting case as 37 years elapsed since the diagnosis of dermatomyositis before the lymphoma has started. We could not state a definite relation between the myositis and the lymphoma but the increased frequency of autoimmune diseases is probably due to immune regulatory problems, that have eventually led to the appearance of the rapidly growing indolent lymphoma.]

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Lege Artis Medicinae

[Report on the 1st Debrecen Medical Days]

BRÚGÓS Boglárka, JUHÁSZ Mária

Lege Artis Medicinae

[THE PSYCHO-SOCIAL BACKGROUND FACTORS OF INDUCED ABORTIONS BASED ON A REPRESENTATIVE NATIONAL SURVEY]

GYŐRFFY Zsuzsa, LŐRINCZ Jenő, ÁDÁM Szilvia, KOPP Mária

[INTRODUCTION - In Hungary the number of induced abortions is three times higher than in European Union countries. The exploration of the bio-psycho-social factors in the background of abortions may serve as an instrument to develop effective prevention. METHODS - The Hungarostudy 2002 national representative study included 12,634 interviewed subjects. The sample represents the Hungarian population above the age of 18, according to gender, age and geographical location. The aim of the health survey was to analyse the physical and psychological status of the Hungarian population as well as the psychosocial risk factors. The prevalence of abortion in the female sub-sample was 22%. We analysed the background factors of abortion with the help of a statistical analysis. RESULTS - The risk factors behind abortions can be grouped as: physical abuse on behalf of partners and/or parents, attachment disorder and early traumatisation, lack of social support, low financial status and inadequate social environment. These factors have all shown significant connection to abortions. Suicide attempts and suicidal thoughts, high Beck Depression (BDI) scores, smoking and alcohol consumption are also significant among those who had abortions. CONCLUSION - An important aim of the Hungarostudy 2002 survey and the follow-ups is to serve a more extensive and effective prevention by exploring the background factors of induced abortions.]

Lege Artis Medicinae

[ENDOTHELIUM PROTECTION IN HYPERTENSIVE PATIENTS]

KÉKES Ede

[The author presents a review about the fundamental principles of normal endothelial function and the main causes for the development of endothelial dysfunction. First of all, endothelial dysfunction results in structural alterations in the wall of the vessel - the „vascular remodelling” in hypertension - and that is the base of hypertensive microangiopathy and target organ damage. In patients with high blood pressure the reactivity of vessels is impaired by the endothelial dysfunction. The members of the main drug groups can influence the vasoactive factors produced in endothelium differently. This different effects of drugs create different clinical benefits. Especially, the ACE inhibitors, the calcium antagonists (mostly the new generation of dihydropyridines) have endothelial protective effects, but some β-blockers, α-1 adrenergic blockers and - in a totally different way of action - the statins are capable of influencing the endothelial dysfunction.]

Lege Artis Medicinae

[HPS]

MATOS Lajos

Lege Artis Medicinae

[THE GENETICS OF DIABETES MELLITUS]

KORÁNYI LÁSZLÓ, PÁNCZÉL Pál

[The number of diabetic patients will be doubled in the coming decades reaching 300 million for year 2025. The number of type 1 diabetics will also be increased but the majority of it will result from the increased number of type 2 diabetics. All types of diabetes are the consequence of a combination of genetic susceptibility and environmental factors, meaning that the prevention of diabetes epidemic cannot be done without the clarification of the genetic background. Significant progression has happened in the discovery of the genetic background of type 1 diabetes mellitus. It was helped by the etiologic classification of the disease: with the new classification the patient groups became more homogeneous. The HLA system is responsible for about 50-70% of the genetic risk while the effects of other genetic factors contribute 1-2% of the genetic susceptibility, respectively. Presently 25 gene regions are known as the different genetic factors of type 1 diabetes mellitus. Regarding the HLA system, the genes and pathomechanism causing the disease are not known. The classification of diabetes mellitus can be based on the HLA type while the predictability of type 1 diabetes mellitus is helped by the HLA type and the INS-VNTR. Much less is known about the genetic background of the polygenic type 2 diabetes mellitus. Its manifestation is now happening at younger age before. The best-fit genetic model consists of only a few genes with moderate effect superimposed on a polygenic background. Several „candidate” genes participating in the impaired insulin secretion and insulin action have already been investigated as the genes responsible for type 2 diabetes. These data showed the specificity in the population and most showed mild or modest association with the disease. Genomewide scans have resulted a number of significant diabetes susceptibility genes specific for a variety of populations, but these investigations have only resulted in the isolation of one gene (calpain 10) that is thought to contribute to type 2 diabetes. Most recent genomewide scans found loci on chromosome 20 in two different populations with significant segregation of type 2 diabetes. These loci are near to the region harboring the transcription factor hepatocyte nuclear factor genes. The transcription regulator HNF family is responsible for the regulation of the expression of several genes participating in the function of liver and pancreatic islet becoming a strong candidate for being a diabetes gene.]

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Lege Artis Medicinae

[Necrotising autoimmune myopathy showing dermatomyositis symptoms during persistent statin treatment]

TIHANYI László, SÜTŐ Gábor, VERESS Gábor

[INTRODUCTION - Dermatomyositis is an idiopathic autoimmune disease. Diagnosis has to be confirmed by biopsy, because clinical symptoms can be similar to those in other myopathies. CASE REPORT - The authors describe the development of dermatomyositis in a 59- year-old man. The patient had a heart attack in 1988. He had been taking simvastatin for three years and atorvastatin since 2002. In June 2008 he complained of myalgia and weakness, became physically very inactive but visited our specialist only half a year later. Laboratory examinations showed a substantial increase of CK, CKMB, LDH and HBDH with troponin T positivity (troponin I was negative). A subsequent biopsy confirmed dermatomyositis. Treatment with azatioprine and methylprednisolone and suspending statin therapy resulted in the regression of his clinical symptoms, his muscles gradually became stronger and his laboratory values normalised. CONCLUSION - During statin treatment in 5% of the cases myopathy can occur, which might develop into amore severe inflammatory disorder.]

Clinical Neuroscience

[Myasthenia in a patient with sarcoidosis and schizophrenia (in English language)]

RÓZSA Csilla, KIS Gábor, KOMOLY Sámuel

[A 44-year-old male patient was hospitalised with paranoid schizophrenia in 1985. Depot neuroleptic treatment was started which successfully prevented further psychotic relapses for the next ten years. His myasthenia gravis started with bulbar signs in 1997 and the symptoms soon became generalized. The diagnosis of myasthenia gravis was confirmed by electromyography, by positive anticholinesterase test and by the detection of anti-acetylcholine receptor antibodies in the serum. Mediastinal CT examination showed enlarged hilar lymph nodes on the left but no thymic pathology was observed. Mediastinoscopy was performed and biopsies were obtained from the affected nodes. Histology revealed sarcoidosis. The patient suffered respiratory crisis following the thoracic intervention (in September 1998). Combined oral corticosteroid (64 mg methylprednisolone/e.o.d.) and azathioprine (150 mg/day) treatment regimen was initiated and complete remission took place in both the myasthenic symptoms and the sarcoidosis. The follow-up CT scans showed no mediastinal pathology (January 2000). During steroid treatment a transient psychotic relapse occured which was successfully managed by supplemental haloperidol medication added to his regular depot neuroleptics. The patient currently takes 150 mg/day azathioprine and receives 40 mg/month flupentixol depot im. His physical and mental status are stable and he has been completely symptome free in the last 24 months. The association of myasthenia gravis and sarcoidosis is very rare. To our best knowledge no case has been reported of a patient suffering from myasthenia gravis, sarcoidosis, and schizophrenia at the same time.]

Lege Artis Medicinae

[ACUTE DERMATOMYOSITIS ASSOCIATED TO THE CARCINOMA OF THE PROSTATE]

TÁLLAI Béla, MORSHED Ali Salah, FLASKÓ Tibor, PONYI Andrea, DANKÓ Katalin, TÓTH Csaba

[INTRODUCTION - In some cases of polymyositis/ dermatomyositis (PM/DM) of autoimmune origin, different malignant tumours can initiate the difficult cascade mechanisms at cell level leading to the rapid weakness of the skeletal muscles. Till now, in the international literature only four cases of PM/DM associated with cancer of prostate has been reported. CASE REPORT - Authors present a case of a 57 yearold male patient, where weakness in patient's movements developed leading to total immobility in 3 months. Purple discoloration developed on his hand and face. Significantly elevated creatin kinase (CK) levels and blood sediment rate with mild anaemia were observed during laboratory examinations. Dysphagia and lack of appetite resulted in the loss 10 kgs in body weight. Both clinical evaluation, elevated serum CK level, skin symptomes, positive electromyography and muscle biopsy confirmed acute definitive dermatomyositis. Urological examination revealed a palpable hard area at the right lobe of the prostate. Prostate biopsy confirmed the presence of carcinoma in the right lobe of the prostate. There was no sign or symptome referring to either local propagation or distant manifestation. Therefore, radical prostatectomy was performed, the tumorous prostate and both seminal vesicles were removed. Histological examination proved malignant focus in the right lobe of the prostate. After the operation patient gradually became stronger, corticosteroid medication were decreased then stopped. Patient's original muscle power and movement recovered and his previous body weight was regained. During the regular control examinations all results of laboratory tests are in normal range. CONCLUSION - In the background of some autoimmune diseases malignant tumours can be revealed. It is rare when urological neoplasms initiative the process. In men with PM/DM commencing beyond 50 years of age it is necessary to think of the presence of prostate cancer, which can be cured by performing radical operation in appropriate time.]

Hungarian Immunology

[Investigation of activated T-cells by non-Hodgkin’s lymphoma patients]

VÁRÓCZY László, GERGELY Lajos, ALEKSZA Magdolna, MILTÉNYI Zsófia, ILLÉS Árpád

[BACKGROUND - The immune system has several mechanisms to fight against developing malignant cell clones in the host, one of them is the activated T-cell response. Both CD4+ helper and CD8+ cytotoxic T-cells bear CD69 and HLA-DR molecules as important surface activation markers. AIM - Our aim was to determine, how the ratio of activated T-cells change in the peripheral blood of non-Hodgkin-lymphoma patients during the periods of polychemotherapy. PATIENTS AND METHODS - We used the peripheral blood samples of 43 non-Hodgkin-lymphoma’s patients (20 females, 23 males, mean age 52.4 years). We determined the level of CD3+/HLADR+ and CD3+/CD69+ T-cell subsets before, during and after the periods of polychemotherapy, using the methods of immunofluorescence stain and flow cytometry. RESULTS - We found the ratio of CD3+/HLA-DR+ cells significantly higher in non-Hodgkin-lymphoma’s patients before treatment compared to healthy controls (10.63% vs. 2.97%, p<0.001). During the period of polychemotherapy, this ratio began to increase significantly (16.94% vs. 10.63%, p=0.006). The level of CD3+/CD69+ cells did not change significantly. After treatment, the ratio of activated T-cells decreased, however, we detected significantly higher rate of CD3+/HLA-DR+ lymphocytes in patients who relapsed within one year than in those who stayed in remission (9.55% vs. 20.62%, p<0.001). CONCLUSION - Investigation of CD3+/HLA-DR+ activated T-cells might be a promising method to determine the immune defence and this way the prognostics of lymphoma patients.]

Lege Artis Medicinae

[NEW DATA ABOUT INFLAMMATORY MYOPATHIES]

DANKÓ Katalin, PONYI Andrea, CONSTANTIN Tamás

[Idiopathic inflammatory myopathies are systemic autoimmune diseases with an immune-mediated inflammation of the striated muscles which lead to progressive muscle weakness. Their cause is still unknown, but recently the understanding of the molecular immunopathology has improved, which may as well offer therapeutic targets in the future. The aim of this review is to present currently available data on the most important factors and processes that are involved in the pathogenesis of these diseases. Although glucocorticoids remain the cornerstone of the treatment for the major forms of idiopathic inflammatory myopathies, there are other efficacious immunosuppressive agents with fewer side effects. The authors discuss classic treatment regimens as well as more recent therapeutic approaches.]