Lege Artis Medicinae

[Plasmocytoid myoepithelioma of the parotid gland]

HAJDU Mária, KRUTSAY Miklós

JUNE 20, 2013

Lege Artis Medicinae - 2013;23(05-06)

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Clinical Neuroscience

A rare entity of acquired idiopathic generalised anhidrosis which has been successfully treated with pulse steroid therapy: Does the histopathology predict the treatment response?

ÖKTEM Özdemir Ece, ÇANKAYA Şeyda, UYKUR Burak Abdullah, ERDEN Simsek Nazan, YULUG Burak

Acquired idiopathic generalised anhidrosis is an uncommon sweating disorder characterized by loss of sweating in the absence of any neurologic, metabolic or sweat gland abnormalities. Although some possible immunological and structural mechanisms have been proposed for this rare entity, the definitive pathophysiology is still un­clear. Despite some successfully treated cases with systemic corticosteroid application, the dose and route of steroid application are controversial. Here, we present a 41-year-old man with lack of genera­lised sweating who has been successfully treated with high dose pulse intravenous prednisolone. We have discussed his clinical and histopathological findings as well as the treatment options in view of the current literature.

Clinical Neuroscience

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.

Lege Artis Medicinae

[Sudden death of a patient with purpura - post mortem recognized eosinophilic granulomatosis with polyangiitis]

DOBREAN Noémi, HAJNAL-PAPP Rozália, TUSA Magdolna, OROJÁN Iván, CSERNI Gábor

[INTRODUCTION - Systemic diseases may sometimes be challenging because physicians do not think about synthesizing the parts to a single entity. CASE REPORT - A 49-year-old asthmatic female was admitted to hospital for the investigation of her cutaneous symptoms suggestive of vasculitis associated with diffuse joint complaints. The chest X-ray raised the possibility of pneumonia or neoplastic disease. Following an episode of chest pain relieved by a non-steroidal anti-inflammatory drug, she suddenly died. Her previous history included restrictive cardiomyopathy, insufficiency of both atrioventricular valves and long dating eosinophilia. Autopsy revealed a partly granulomatous eosinophilic inflammatory process in several organs, including the heart, the lungs, the kidneys, the colon and the pituitary gland. Retrospective collection of unknown anamnestic features and symptoms made possible to unify the pieces of information and symptoms to a single entity, the Churg-Strauss syndrome (eosinophilic granulomatosis with polyangiitis, EGPA). CONCLUSIONS - Bronchial asthma seldom leads to death. It can rarely be part of the Churg-Strauss syndrome, of which the manifestation may be related to the administration of leukotriene antagonists also used in the presented case. These drugs may allow the withdrawal of systemic steroid therapy which is beneficial not only in the treatment of asthma but also of the syndrome. Lowering the dose of steroids may promote the development of the full blown pattern of the latter.]

Hungarian Immunology

[Autoimmun thyroiditis presence in patient with Hodgkin’s disease in remission]

BÍRÓ Edit, BAKÓ Gyula, SZEGEDI Gyula, ILLÉS Árpád

[INTRODUCTION - It is known that the incidence of hypothyroidism is higher in long term survivor patients with Hodgkin's disease, and it is supposed to be the result of treatment, such as neck radiotherapy. The author believe that other etiologic factors may also play a role in the development of hypothyroidism. PATIENTS AND METHODS - Looking for the possible causes of hypothyroidism, the thyroid function of 151 patients treated for Hodgkin's disease since 1970 were examined. These patients with Hodgkin's disease in complete remission for at least one year and their data on thyroid autoantibody positivity [antithyroid peroxidase antibody (aTPO), antihuman thyroglobulin antibody (aHTG), TSH antireceptor antibody (TRAK)] were analysed. RESULTS - Among the patients with antibody positive 26 received ultrasound scanning and fine needle aspiration cytology of the thyroid, which confirmed autoimmun thyroiditis. There were no significant differences between the mean age, histologic subtypes and stage of the disease between the patients with antibody positive those with antibody negative. A significantly greater number of women in the group of antibody positive patients was found and thyroid dysfunction (two cases of hyper, and 13 cases of hyperthyroidism) was revealed in 53.6% of the patients. Though antibody positivity was more frequent in patients having been treated by neck irradiation, but no significant relationship was found between the form of Hodgkin’s disease treatment and the development of thyroiditis. Thus the authors cannot confirm the assumption according to which the autoantigens released from the thyroid gland damaged by neck irradiation for Hodgkin's disease would provoke the development of thyroiditis. Since - independently of the type of treatment received - the incidence of thyroiditis is higher in patients with Hodgkin's disease, it is probable that immune regulation disorders may also play a role in its development and thus hypothyroidism is the result of a multi-factor process. DISCUSSION - These results underline the importance of a regular control of thyroid hormones and thyroid autoantibodies in follow up Hodgkin’s disease patients. Levothyroxine administered as an isohormone treatment may inhibit the development of hypothyroidism in patients with thyroiditis may improve the quality of their life.]

Hypertension and nephrology

[The role of vitamin D receptor and the risk reducing effect of vitamin D receptor agonists in chronic kidney disease]

KISS István, KULCSÁR Imre, BARABÁS Noémi, KERKOVITS Lóránt

[Vitamin D3 is produced in the skin and is modified in the liver and kidney to the active metabolite form, 1,25-dyhydroxyvitamin D3 (calcitriol). Calcitriol binds to a nuclear receptor, the vitamin D receptor (VDR), and activates processes that bind to vitamin D. The classical effects of vitamin D receptor activator or agonist (VDRA) therapy for the treatment of secondary hyperparathyroidism in patients with chronic kidney disease primarily involves suppressive effects on the parathyroid gland, and regulation of calcium and phosphorus absorption in the intestine an mobilization in bone. Several VDR agonists have been developed for the treatment of osteoporosis, hyperparathyroidism secondary to chronic kidney disease (CKD), and psoriasis. Secondary hyperparathyroidism (SHPT) is a common and serious consequence of CKD. SHPT is a complex condition characterized by a decline in 1,25-dihidroxi vitamin D and consequent VDR activation, abnormalities in serum calcium and phosphorus levels, parathyroid gland hyperplasia, elevated parathyroid hormone (PTH) secretion, and systemic mineral and bone abnormalities. There are three classes of drug used for treatment of SHPT (non-selective and selective VDR activators, and calcimimetics). Observational studies in hemodialysis patients report improved cardiovascular and allcause survival among those received VDRA therapy compared with those not on VDRA therapy. The survival benefits of selective VDRA paricalcitol appear to be linked to "non classical" action of VDRA, possibly through VDRA-mediated modulation of gene expression. VRDAs are reported to have beneficial effects such as anti-inflammatory and antithrombotic effects, inhibition of vascular calcification and stiffening, inhibition of vascular smooth muscle cell proliferation, and regression of left ventricular hypertrophy. VDRA are also reported to negatively regulate the renin-angiotensin system, which plays a key role in hypertension, myocardial infarction and stroke. Data from epidemiological, preclinical and clinical studies have shown that vitamin D and/or 25(OH) vitamin D deficiency is associated with increased risk for cardiovascular disease (CVD). The selective VDR agonists are associated direct protective effects on glomerular architecture and antiproteinuric effects in response to renal damage. Emerging evidence suggest that VDR plays important roles in modulating cardiovascular, immunological, metabolic and other function. Paricalcitol may prove to have a substantial beneficial effect on cardiac disease and its outcome in patients with CKD.]