Interesting case of ovarian sarcoidosis: The value of multi disciplinary team working

BACKGROUND: Sarcoidosis of the genital tract is a rare condition. Ovarian manifestation of this disease is rarer still. CASE PRESENTATION: The case presented here represents ovarian manifestation of sarcoidosis. At the point of referral to our hospital, based on computerised tomography (CT) ovarian carcinoma was a differential diagnosis. Further magnetic resonance imaging along with CT guided biopsy aided by laboratory study supported a diagnosis of sarcoidosis. Patient responded to medical management by a multidisciplinary team. CONCLUSION: The case shows the importance of FNAC and biopsy in case or ovarian masses and multi disciplinary team approach to management

Σημασία της κυτταρικής εξειδίκευσης και συσχέτιση αυτής με τη δράση βιολογικών τροποποιητών: In vitro μελέτη σε όρους πασχόντων από συμπαγή νεοπλάσματα

Cell to cell communication, positive and negative influence of normal and abnormal biological regulators and reciprocal cytokine interactions, are now recognized to play an important role in modulating many pathophysiological conditions. Cytokines, are regulatory proteins, produced by virtually any cell of the multicellular organisms, having pleiotropic regulatory effects in many cell types. Among cytokines, IFN family is in the forefront of biomedical research, although it is known that many types of neoplasia poorly respond in IFN treatment, or at least in an unpredictable manner. Moreover, natural inhibitors to various cytokines and IFNs have been documented in several in vitro systems, as well as in vivo. Especially interferon inhibitors have been found in a variety of tissues, body fluids, serum and many types of cells. Inactivation and inhibition of IFN action by a number of factors have been implicated for the IFN ineffectiveness, during IFN treatment of patients with malignant neoplasia. So our efforts were confined to monitoring IFN-blocking activity, in serum samples of several groups of human malignancies, before any kind of treatment. Aim of this study was to investigate the implication of ceil or tissue specificity, in relation to IFN-blocking activity of sera from patients with solid tumors, in 4 epithelial cell lines: a human cell line originated from lung cancer A₅₄₉, a human embryonic line-Intestine 407, a cell line derived from human amnion tissue-Wish and the Chang-Liver cells, originated from healthy human liver. An IFN-bioassay was used for the measurements of serum IFN-like and IFN-blocking activity. In brief, the presence of endogenous IFN and IFN inhibitors was determined by assaying the inhibition of the virus specific cytopathic effect (cpe) of the vesicular stomatitis virus, in the 4 forementioned cell lines, which they have already been exposed either to a serum sample (measurement of endogenous IFN), or to the mixture of serum and diluted IFN (measurement of IFN inhibitors). There was no endogenous IFN in any of the patients. Concerning the IFN blocking activity of sera, a marked variability is prominent, depended on the type of neoplasia. From the tested lines, A₅₄₉ cells respond more efficiently to serum IFN-blocking activity. The other two ceil lines (intestine-407 and Chang-Liver) respond poorly and the fourth one (Wish) does not respond at all. The presence of interferon blocking activity in cancer patients, may contribute to their variable response to interferon treatment. The correlation between the IFN inhibitors' activity and the cell specificity is evident, but its clinical significance needs further investigation.Ο διαφορετικός βαθμός ή και τρόπος ανταπόκρισης των διαφόρων τύπων κυττάρων στη δράση βιολογικών τροποποιητών απετέλεσε το θέμα της παρούσης διδακτορικής διατριβής. Οι βιολογικοί τροποποιητές (ιντερλευκίνες, ιντερφερόνες, αυξητικοί παράγοντες κ.ά.) συνιστούν φυσικά προϊόντα του ευκαρυωτικού κυττάρου και ελέγχουν το σύνολο των κυτταρικών αλληλεπιδράσεων, αποτελούντες το γνωστό δίκτυο των κυτοκινών. Μεταξύ των κυτοκινών, η οικογένεια των ιντερφερονών ευρίσκεται σήμερα στο επίκεντρο της βιολογικής και ιατρικής έρευνας, υποσχόμενη μία περισσότερο αποτελεσματική θεραπευτική προσέγγιση σε διαφόρους τύπους νεοπλασιών. Παράλληλα, η ύπαρξη ουσιών που ανταγωνίζονται ή αναστέλλουν τη δράση της IFN, αποτέλεσε αντικείμενο πολλών μελετών τα τελευταία χρόνια. Μάλιστα η παρουσία των ουσιών αυτών σε υγρά του σώματος και μάλιστα στον ορό, αποτέλεσε μια προσιτή πηγή υλικού που διευκόλυνε τη μελέτη τους. Η δράση της IFN και των αναστολέων της σε κυτταρικό επίπεδο δεν αποτελεί μια στατική λειτουργία. Είναι μια δυναμική σχέση, η διερεύνηση της οποίας στηρίζεται σε βιολογικές μεθόδους ανίχνευσης, που χρησιμοποιούν εξειδικευμένες καλλιέργειες κυττάρων (πρότυπες κυτταρικές σειρές που εξασφαλίζουν συνθήκες παρόμοιες με εκείνες του κυτταρικού μικροπεριβάλλοντος). Τέτοιου τύπου κυτταρικά συστήματα και μάλιστα προερχόμενα από τέσσερα διαφορετικά είδη κυττάρων, χρησιμοποιήθηκαν στη παρούσα μελέτη. Έτσι διερευνήθηκε η σημασία της κυτταρικής εξειδίκευσης και η συσχέτιση αυτής με τη δράση βιολογικών τροποποιητών (IFN και αναστολείς αυτής), σε περιπτώσεις νεοπλασιών και ειδικότερα συμπαγών όγκων. Οι προσδιορισμοί της IFN βασίστηκαν στην αναστολή ανάπτυξης της κυτταροπαθογόνου δράσης ιού αναφοράς σε ευαίσθητες καλλιέργειες κυττάρων, που είχαν εκτεθεί στη δράση δείγματος ορού, που ελέγχεται για τη παρουσία ενδογενούς IFN. Οι προσδιορισμοί των αναστολέων έναντι της IFN βασίστηκαν στην ίδια αρχή, δηλαδή τον ανταγωνισμό της αναστολής του ιού που προκαλεί μία δεδομένη ποσότητα ιντερφερόνης. Η παρουσία ενδογενούς IFN στους εξετασθέντες ορούς βρέθηκε σε ποσοστό πρακτικά αμελητέο. Όσον αφορά την παρουσία αναστολέων, τα αποτελέσματα έδειξαν ευρεία διακύμανση της συχνότητας των ορών που αναστέλλουν την αντιική δράση της ιντερφερόνης, σχετιζόμενη αφ’ ενός με τον τύπο της νεοπλασίας εκ της οποίας προέρχεται ο ορός και αφ’ ετέρου με τον τύπο των κυττάρων των καλλιεργειών που χρησιμοποιήθηκαν. Η διακύμανση που παρατηρήθηκε στα αποτελέσματα προσδιορισμού των αναστολέων της IFN κατά νεοπλασία, ίσως αποτελέσει μία ικανοποιητική εξήγηση για τα σαφώς αντιφατικά αποτελέσματα που παρουσιάζει η κλινική της εφαρμογή. Όσον αφορά τη κλινική σημασία της αποδειχθείσης στη μελέτη ύπαρξης εμφανούς συσχέτισης μεταξύ της κυτταρικής εξειδίκευσης και της δράσης της ιντερφερόνης και των αναστολέων της, αυτή θα αποτελέσει οπωσδήποτε αντικείμενο περαιτέρω διερεύνησης

Cutaneous manifestations in relation to immunologic parameters in a cohort of primary myelodysplastic syndrome patients

Background: Cutaneous lesions in myelodysplastic syndrome (MDS) may be specific or not and may reveal bone marrow transformation. Our purpose was to investigate in a cohort of 84 MDS patients the correlation of cutaneous findings with immunologic parameters and prognostic features of MDS in order to clarify their potential clinical significance. Materials and methods: We studied a cohort of 84 newly diagnosed MDS patients in order to assess the cutaneous findings present at the time of diagnosis and during 1 to 3.years of follow-up. We described the clinical variety of cutaneous findings ascertained by histology. We also looked for any association between the group of MDS patients with skin manifestations and MDS subtype, immunologic and prognostic features highlighting transformation to acute leukaemia. Results: Twenty-one patients presented cutaneous manifestations: 1 patient developed leukaemia cutis, 6 patients photosensitivity not associated with autoimmune disease, 3 prurigo nodularis, 2 Sweet's syndrome, 6 leucocytoclastic vasculitis, 2 ecchymoses and purpura associated with preexisting relapsing polychondritis, 1 patient subcutaneous nodules associated with Wegener's granulomatosis and 1 patient with malar rash and oral ulcers associated with preexisting systemic lupus erythematosus. Adjusted for age and gender, the presence of skin findings constitutes a significant predictor of the high-risk MDS subgroup (odds ratio, 3.59; 95% confidence interval, 1.18-10.92). Hypergammaglobulinemia was significantly higher in the MDS subgroup with skin manifestations (P = 0.03). Conclusion: Most MDS patients with cutaneous manifestations belong to the high-risk MDS subgroup and present hypergammaglobulinemia. Early biopsy of skin lesions in myelodysplasia is indicated. © 2007 The Authors Journal compilation © 2007 European Academy of Dermatology and Venereology

Hypocalcemia, hypomagnesemia, and hypokalemia following hydrofluoric acid chemical injury

Dermal exposure to hydrofluoric acid could potentially result in severe serum calcium and magnesium depletion induced by binding with fluoride anion. This report describes the case of a 48-year-old man who developed hypocalcemia and hypomagnesemia accompanied by hypokalemia-an interesting finding-following a chemical injury with exposure to 70% hydrofluoric acid. Successful treatment included administration of calcium gluconate and magnesium both intravenously and topically. © 2008 The American Burn Association

Is thyroid autoimmunity a risk factor for developing primary myelodysplastic syndrome?

Objective: Thyroid disease has been associated with leukemia and lymphoma. No previous study using clinical and laboratory data has explored whether thyroid disease and especially autoimmune thyroid disease (ATD) is associated with myelodysplastic syndrome (MDS) risk. In this case-control study, we investigated the association of ATD with MDS. Methods: Our study included 101 cases with incident primary MDS confirmed by histology and cytogenetics, and 101 controls matched on gender and age, admitted for non-neoplastic and non-infectious diseases. All subjects were submitted to clinical, ultrasound thyroid evaluation and serum free T3, free T4, TSH, thyroglobulin, and thyroperoxidase antibodies determination. Results: Adjusting for age, gender, and body mass index, there was statistically significant evidence that ATD is associated with increased risk of MDS (OR = 2.58, 95% CI 1.29-5.16). Interestingly, ATD starting from the remote past (more than 10 years from MDS onset) was positively associated with MDS risk (OR = 5.73. 95% CI 2.03-16.16). Mean serum levels of fT3, fT4, and thyroid antibodies were significantly higher in MDS patients and mean TSH serum levels were significantly lower in MDS patients than in controls (p < 0.05). Conclusion: Biological plausibility and empirical evidence highlights the importance of ATD in MDS etiopathogenesis. Further studies are needed to explore underlying mechanisms associating thyroid autoimmunity with leukemogenesis. © 2007 Springer Science+Business Media B.V

Association of thyroid disease and thyroid autoimmunity with multiple myeloma risk: A case-control study

Thyroid disease has been associated with lymphohematopoietic cancer (LHC). No previous study using clinical, sonographic and laboratory data has explored whether thyroid disease and specifically autoimmune thyroid disease (ATD) is associated with multiple myeloma (MM) risk. 73 patients with incident primary MM and 73 hospital controls admitted for non-neoplastic and non-infectious conditions, matched on gender and age were studied between 2001 and 2007. Blood samples were collected. All subjects were submitted to clinical, ultrasound and laboratory thyroid evaluation. The prevalence of clinical thyroid disease in MM patients was significantly higher than in controls (p = 0.002). ATD was associated with increased risk of MM, adjusting for age, gender, body mass index and familial history of LHC [OR = 5.68, 95% confidence interval (CI): 1.69-19.13]. Controlling for the above variables, an individual suffering from any thyroid disease more than 10 years has about 2.41 times more likely the risk to develop MM than an individual without any thyroid disease (OR = 2.41, 95% CI: 1.35-4.29). Also, adjusting for age, gender, BMI and family history of LHC, a familial history of thyroid disease is associated with increased risk of MM (OR = 3.23, 95% CI: 1.25-8.31). Further studies are needed to explore underlying mechanisms associating thyroid autoimmunity with plasma cell transformation