Lege Artis Medicinae

[Adenoma of the adrenal cortex imitating renal cancer]

SEJBEN István, GÖCZŐ Katalin, SZABÓ Zoltán, CSERNI Gábor

FEBRUARY 21, 2008

Lege Artis Medicinae - 2008;18(02)

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Life threatening rare lymphomas presenting as longitudinally extensive transverse myelitis: a diagnostic challenge

TOLVAJ Balázs, HAHN Katalin, NAGY Zsuzsanna, VADVÁRI Árpád, CSOMOR Judit, GELPI Ellen, ILLÉS Zsolt, GARZULY Ferenc

Background and aims – Description of two cases of rare intravascular large B-cell lymphoma and secondary T-cell lymphoma diagnosed postmortem, that manifested clinically as longitudinally extensive transverse myelitis (LETM). We discuss causes of diagnostic difficulties, deceptive radiological and histological investigations, and outline diagnostic procedures based on our and previously reported cases. Case reports – Our first case, a 48-year-old female was admitted to the neurological department due to paraparesis. MRI suggested LETM, but the treatments were ineffective. She died after four weeks because of pneumonia and untreatable polyserositis. Pathological examination revealed intravascular large B-cell lymphoma (IVL). Our second case, a 61-year-old man presented with headache and paraparesis. MRI showed small bitemporal lesions and lesions suggesting LETM. Diagnostic investigations were unsuccessful, including tests for possible lymphoma (CSF flow cytometry and muscle biopsy for suspected IVL). Chest CT showed focal inflammation in a small area of the lung, and adrenal adenoma. Brain biopsy sample from the affected temporal area suggested T-cell mediated lymphocytic (paraneoplastic or viral) meningoencephalitis and excluded diffuse large B-cell lymphoma. The symptoms worsened, and the patient died in the sixth week of disease. The pathological examination of the presumed adenoma in the adrenal gland, the pancreatic tail and the lung lesions revealed peripheral T-cell lymphoma, as did the brain and spinal cord lesions. Even at histological examination, the T-cell lymphoma had the misleading appearance of inflammatory condition as did the MRI. Conclusion – Lymphoma can manifest as LETM. In cases of etiologically unclear atypical LETM in patients older than 40 years, a random skin biopsy (with subcutaneous adipose tissue) from the thigh and from the abdomen is strongly recommended as soon as possible. This may detect IVL and provide the possibility of prompt chemotherapy. In case of suspicion of lymphoma, parallel examination of the CSF by flow cytometry is also recommended. If skin biopsy is negative but lymphoma suspicion remains high, biopsy from other sites (bone marrow, lymph nodes or adrenal gland lesion) or from a simultaneously existing cerebral lesion is suggested, to exclude or prove diffuse large B-cell lymphoma, IVL, or a rare T-cell lymphoma.

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DOLGOS Szilveszter, TÁRNOKI Ádám Domonkos, TÁRNOKI Dávid László

[The most common monogenic nephropathy is a congenital, cystic, bulky process in the kidney that leads to a gradual deterioration in renal function. Renal failure is often associated with cystic liver or pancreatic lesions, cerebral artery aneurysm, or mitral prolapse.]

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[Calcium ion is a common denominator in the pathophysiological processes of amyotrophic lateral sclerosis]

PATAI Roland, NÓGRÁDI Bernát, MESZLÉNYI Valéria, OBÁL Izabella, ENGELHARDT József István, SIKLÓS László

[Amyotrophic lateral sclerosis (ALS), the most frequent motor neuron disease is characterized by progressive muscle weakness caused by the degeneration of the motor neurons in the spinal cord and motor cortex. However, according to the recent observations, ALS is a rather complex syndrome which frequently involves symptoms of cognitive impairment. Therefore, ALS cases can be interpreted in a clinico-pathological spectrum spanning from the classical ALS involving only the motor system to the fronto-temporal dementia. The progression of the disease, however, manifested in the degeneration of the upper and lower motor neurons, is based on the same complex pathobiology. The main elements of the pathomechanism, such as oxidative stress, excitotoxicity, immune/inflammatory processes and mitochondrial dysfunction are well described already, which operate in orchestrated way and amplify the deleterious effect of each other. It is assumed that calcium ions act as a catalyst in this interaction, hence each of the individual mechanisms has strong, positive and reciprocal calcium dependence thus may combine the individual pathological processes into a unified escalating mechanism of neuronal destruction. This review provides an overview of the role of calcium in connecting and amplifying the major mechanisms which lead to degeneration of the motor neurons in ALS. ]

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[Health behaviour and cancer screening]

DÖBRŐSSY Lajos, KOVÁCS Attila, CORNIDES Ágnes, BUDAI András

[The purpose of the paper is to find out: what influences the participation in cancer screening. For answer, concepts such as health, health consciousness, health culture, health behaviour have been taken into account, and the factors that have an influence on the health behaviour considered. It concludes that the participation in screening makes a part of the health behaviour which is a mandatory task for the health education to include screening into the health message. ]

Lege Artis Medicinae

[THE ROLE OF REGISTRY ANALYSIS IN THE FORMING OF THERAPEUTIC GUIDELINES IN RENAL TRANSPLANTATION]

PERNER Ferenc, HERCZEG Balázs, SZENOHRADSZKY Pál, ASZTALOS László, KALMÁR-NAGY Károly, JÁRAY Jenő

[The authors assess the two main outcomes of the immunosuppressive therapy after renal transplantation: graft and patient survival. According to their view, evidence from randomised clinical trials results can be well complemented by the several unique transplant registries and outcome research based upon these databases. The comparison of evidence from these two sources addresses the question of achievable outcome under ideal (controlled) versus real life conditions. Based on a systematic review of the relevant clinical trials and registries it can be stated, that in the case of some immunosuppressants (mycophenolate mofetil vs azathioprine, microemulsified cyclosporin vs cyclosporin and tacrolimus vs cyclosporin) the improvement in the intermediate outcome can lead to improved graft and patient survival, while in the case of other drugs no significant difference in hard endpoints were detected (tacrolimus vs microemulsified cyclosporin). Evidence on graft and patient survival differences could not be derived from traditional randomised clinical trials, only from transplant registries. For the sake of improved evidence based therapeutic guidelines in renal transplantation, authors call for further development of the Hungarian transplant registry.]