Mounier-Kuhn Syndrome in an Elderly Female with Pulmonary Fibrosis

Mounier-Kuhn syndrome (MKS), or tracheobronchomegaly, is a rare clinical and radiologic condition characterized by pronounced tracheobronchial dilation and recurrent lower respiratory tract infections. Tracheobronchomegaly presents when the defect extends to the central bronchi. MKS can be diagnosed in adult women when the transverse and sagittal diameters of the trachea, right mainstem bronchus, and left mainstem bronchus exceed 21, 23, 19.8, and 17.4 mm, respectively. Its diagnosis is based on chest radiograph and chest computed tomography (CT). Patients, usually middle-aged men, may be asymptomatic or present with clinical manifestations ranging from minimal symptoms with preserved lung function to severe respiratory failure. Pulmonary function tests (PFTs) typically reveal a restrictive pattern. This report presents an elderly woman with previously diagnosed pulmonary fibrosis with symptoms of increased sputum production and haemoptysis. High-resolution chest CT showed tracheal and main stem bronchi dilatation along with bronchial diverticulosis. PFTs indicated a restrictive pattern characteristic of the underlying pulmonary fibrosis. The patient is the oldest, referred to the female gender, at presentation of MKS hitherto reported. This case highlights the need to include MKS in the differential diagnosis of recurrent lower respiratory tract infections, even in older subjects

Prevlast CD4 pozitivnih stromalnih stanica koštane srži u bolesnika s ranim stupnjem Hodgkinove bolesti miješane celularnosti

The aim of the study was to determine the possible bone marrow involvement in patients with early stages of classic Hodgkin\u27s disease mixed cellularity variant diagnosed by lymph node biopsy at initial presentation not responding to radiotherapy alone. The study cohort consisted of 20 patients (18 displaying B-cell genotype and two T-cell genotype) with stages I-II Hodgkin\u27s disease according to Ann Arbor classification treated with radiotherapy alone, seven of them not responding to therapy. Southern blot hybridization using a specific EBV Bam H1W fragment probe showed the presence of EBV genomes in two patients. All 20 patients underwent iliac crest trephine biopsy and a panel of antibodies including CD45, CD20, CD4, CD8, CD45RO, CD56, CD30, ALK-1, CD-15, EMA, CD61, and CD68 were performed. A statistically significant predominance of CD45, CD45RO and CD4 positive stromal cells was found in seven patients that failed to respond to therapy (c2-test: p=0.021, p=0.019 and p=0.015, respectively). The predominance of CD4 positive cells in the bone marrow stroma might be suggestive of involvement by Hodgkin\u27s disease in the early stage (I-II) patients (indicating upstaging) who fail to show remission on radiotherapy alone, and could explain the abnormal cytokine production, which may contribute to diminished T-cell immunity and inefficient antitumor responses despite a vast majority of infiltrating reactive immune cells.Cilj rada bio je utvrditi moguću zahvaćenost koštane srži u bolesnika s ranim stadijima klasične Hodgkinove bolesti, varijanta miješane celularnosti, dijagnosticirane biopsijom limfnog čvora, koji pri prvom dolasku nisu odgovorili na samu radioterapiju. Bolesnička skupina sastojala se je od 20 bolesnika (18 s B-staničnim genotipom i dvoje s T-staničnim genotipom) u I.-II. stadiju prema klasifikaciji iz Ann Arbora, koji su bili liječeni samo radioterapijom. Sedmoro bolesnika nije odgovaralo na ovu vrst liječenja. Southern blot hibridizacija uz primjenu specifične fragmentne sonde EBV Bam H1W pokazala je prisutnost EBV genoma u dvoje bolesnika. Svih 20 bolesnika podvrgnuto je trepanacijskoj biopsiji ilijačnog grebena i provedeno je ispitivanje panelom protutijela uključujući CD45, CD20, CD4, CD8, CD45RO, CD56, CD30, ALK-1, CD-15, EMA, CD61 i CD68. U sedmoro bolesnika koji nisu odgovarali na liječenje utvrđena je statistički značajna prevlast stromalnih stanica pozitivnih na CD45, CD45RO i CD4 (c2-test: p=0,021, p=0,019 odnosno p=0,015). Prevlast stanica pozitivnih na CD4 u stromi koštane srži moglo bi ukazivati na zahvaćenost koštane srži Hodgkinovom bolešću u bolesnika s ranim stadijem (I.-II.) (tj. porast stadija) u kojih ne dolazi do remisije bolesti nakon same radioterapije, te bi moglo objasniti nenormalnu proizvodnju citokina, što možda doprinosi smanjenoj T-imunosti i nedostatnom protutumorskom odgovoru usprkos goleme većine infiltriranih reaktivnih imunih stanica

Prevlast CD4 pozitivnih stromalnih stanica koštane srži u bolesnika s ranim stupnjem Hodgkinove bolesti miješane celularnosti

The aim of the study was to determine the possible bone marrow involvement in patients with early stages of classic Hodgkin\u27s disease mixed cellularity variant diagnosed by lymph node biopsy at initial presentation not responding to radiotherapy alone. The study cohort consisted of 20 patients (18 displaying B-cell genotype and two T-cell genotype) with stages I-II Hodgkin\u27s disease according to Ann Arbor classification treated with radiotherapy alone, seven of them not responding to therapy. Southern blot hybridization using a specific EBV Bam H1W fragment probe showed the presence of EBV genomes in two patients. All 20 patients underwent iliac crest trephine biopsy and a panel of antibodies including CD45, CD20, CD4, CD8, CD45RO, CD56, CD30, ALK-1, CD-15, EMA, CD61, and CD68 were performed. A statistically significant predominance of CD45, CD45RO and CD4 positive stromal cells was found in seven patients that failed to respond to therapy (c2-test: p=0.021, p=0.019 and p=0.015, respectively). The predominance of CD4 positive cells in the bone marrow stroma might be suggestive of involvement by Hodgkin\u27s disease in the early stage (I-II) patients (indicating upstaging) who fail to show remission on radiotherapy alone, and could explain the abnormal cytokine production, which may contribute to diminished T-cell immunity and inefficient antitumor responses despite a vast majority of infiltrating reactive immune cells.Cilj rada bio je utvrditi moguću zahvaćenost koštane srži u bolesnika s ranim stadijima klasične Hodgkinove bolesti, varijanta miješane celularnosti, dijagnosticirane biopsijom limfnog čvora, koji pri prvom dolasku nisu odgovorili na samu radioterapiju. Bolesnička skupina sastojala se je od 20 bolesnika (18 s B-staničnim genotipom i dvoje s T-staničnim genotipom) u I.-II. stadiju prema klasifikaciji iz Ann Arbora, koji su bili liječeni samo radioterapijom. Sedmoro bolesnika nije odgovaralo na ovu vrst liječenja. Southern blot hibridizacija uz primjenu specifične fragmentne sonde EBV Bam H1W pokazala je prisutnost EBV genoma u dvoje bolesnika. Svih 20 bolesnika podvrgnuto je trepanacijskoj biopsiji ilijačnog grebena i provedeno je ispitivanje panelom protutijela uključujući CD45, CD20, CD4, CD8, CD45RO, CD56, CD30, ALK-1, CD-15, EMA, CD61 i CD68. U sedmoro bolesnika koji nisu odgovarali na liječenje utvrđena je statistički značajna prevlast stromalnih stanica pozitivnih na CD45, CD45RO i CD4 (c2-test: p=0,021, p=0,019 odnosno p=0,015). Prevlast stanica pozitivnih na CD4 u stromi koštane srži moglo bi ukazivati na zahvaćenost koštane srži Hodgkinovom bolešću u bolesnika s ranim stadijem (I.-II.) (tj. porast stadija) u kojih ne dolazi do remisije bolesti nakon same radioterapije, te bi moglo objasniti nenormalnu proizvodnju citokina, što možda doprinosi smanjenoj T-imunosti i nedostatnom protutumorskom odgovoru usprkos goleme većine infiltriranih reaktivnih imunih stanica

Bronchial-associated lymphoid tissue (BALT) , during fetal development: an immunohistochemical study

Purpose : The aim of this study was to examine and analyse in the lung parenchyma, apart from the appearance of BALT, the expression of structural proteins with a specific role during embryogenesis and growth.Materials and Methods : We examined a series of 149 paraffin-embedded post-mortem human fetal lung specimens at the second trimester of development. Using immunohistochemical methods we examined the expression and distribution of B and T-lymphocytes (CD20 and CD3) in lung parenchyma, as well as the expression of tenascin-c, vimentin and fibronectin in the intermediate cells.Results : The results of this study showed that: 1) The BALT does not develop in fetal period, 2) The BALT which develops during fetal period is probably in response to some antigenic stimulation where in our cases occurs to be changes of chorioamnionitis. The response is depicting with the appearance of scattered lymphocytic infiltrate or ill-defined lymphoid aggregations in lung parenchyma and particularly around the bifurcations of the bronchial tree. 3) In all of our cases in which severe lesions of chorioamnionitis were present, a decreased expression for tenascin-c, vimentin and fibronectin, and an increased expression for CD3 and CD20 in the intermediate cells of lung parenchyma was observed in comparison with the cases in which chorioamnionitis was absent.Conclusion : The expression pattern of intermediate filaments proteins and the expression of lymphoid tissue (BALT) in the lung parenchyma can be modified by the presence of chorioamnionitis in the fetal membranes. Inflammation induced by chorioamnionitis results in increased risk of childhood asthmaΣτόχος : Ο σκοπός της παρούσας μελέτης είναι η εξέταση και η ανάλυση στο πνευμονικό παρέγχυμα τόσο της ύπαρξης του λεμφικού ιστού στο βρογχικό βλεννογόνο (BALT) όσο και της έκφρασης των δομικών πρωτεϊνών με σημαίνοντα ρόλο τόσο κατά τη διάρκεια της εμβρυογένεσης όσο και της ανάπτυξης.Υλικά-Μέθοδοι : Εξετάσαμε 149 έμβρυα ανθρώπου με τους αντίστοιχους υμένες που αποβλήθηκαν αυτόματα ή για θεραπευτικούς σκοπούς. Ελήφθησαν τομές από το πνευμονικό παρέγχυμα και το βρογχικό δέντρο κατά το δεύτερο τρίμηνο της ανάπτυξης και εμβαπτίστηκαν με παραφίνη. Χρησιμοποιώντας ανοσοϊστοχημικές μεθόδους εξετάσαμε την έκφραση και κατανομή των Β και Τ-λεμφοκυττάρων στο πνευμονικό παρέγχυμα, καθώς και την έκφραση της τενασκίνης, βιμεντίνης, ινονεκτίνης στα ενδιάμεσα κύτταρα του πνευμονικού παρεγχύματος.Αποτελέσματα : Τα αποτελέσματα αυτής της μελέτης ανέδειξαν ότι : 1) Το BALT δεν είναι φυσιολογικά παρών κατά την εμβρυϊκή περίοδο, 2) Το BALT που αναπτύσσεται κατά τη διάρκεια της εμβρυϊκής περιόδου οφείλεται σε αντιγονική διέγερση, όπως στη συγκεκριμένη περίπτωση λόγω χοριοαμνιονίτιδας. Η ανταπόκριση στη λοίμωξη αυτή επιβεβαιώνεται με την ύπαρξη διάσπαρτων λεμφικών αθροίσεων –λεμφοζιδίων στους πνεύμονες και στο βρογχικό δέντρο των εμβρύων. 3) Σε όλες τις περιπτώσεις πνευμονικού παρεγχύματος εμβρύων με σοβαρές αλλοιώσεις χοριοαμνιονίτιδας παρατηρήθηκε μείωση της έκφρασης των δομικών πρωτεϊνών, τενασκίνης, βιμεντίνης, ινονεκτίνης και αύξηση της έκφρασης των CD3 και CD20 στα ενδιάμεσα κύτταρα σε σύγκριση με περιπτώσεις εμβρύων χωρίς αλλοιώσεις χοριοαμνιονίτιδας.Συμπεράσματα : Το πρότυπο έκφρασης των δομικών πρωτεϊνών του κυτταροσκελετού του πνευμονικού παρεγχύματος και της έκφρασης του λεμφικού ιστού στο βρογχικό βλεννογόνο (BALT) μπορεί να τροποποιηθεί λόγω της παρουσίας χοριοαμνιονίτιδας. Η εμμένουσα φλεγμονή που προκαλείται από τη χοριοαμνιονίτιδα συσχετίζεται με αυξημένο κίνδυνο δημιουργίας παιδικού βρογχικού άσθματο

Polymorphous Low Grade Adenocarcinoma of the Parotid Gland. Cytological, Histological and Immunohistochemical Features and Review of the Literature

Aim: Polymorphous low grade adenocarcinoma of the salivary glands (PLGA) is a low grade neoplasm that predominantly occurs in the minor salivary glands. In this site is amenable to biopsy and histologic diagnosis. However, experience with cytological findings in these tumors is limited. We describe the cytology of this entity. Experimental design: Touch imprint cytology of a primary parotid PLGA is specified and correlated with histology. Results: Smears were hypercellular showing branching papillae, sheets and clusters of uniform cells with bland nuclei, dispersed chromatin and no nucleoli. The cells had a scant to moderate amount of eosinophilic cytoplasm. They formed tubular structures containing hyaline globules. Conclusions. The cytologic differential diagnosis of PLGA includes adenoid cystic carcinoma, pleomorphic adenoma, and monomorphic adenoma. PLGA should be considered in the differential diagnosis of head and neck tumors, where the cytology suggests on of the above mentioned tumors, even when the clinical findings (involvement of a major salivary gland, lymph node metastasis) is not typical of PLGA

Human Decidual Cells Activity in Women with Spontaneous Abortions of Probable CMV Aetiology During the First Trimester of Gestation. An Immunohistochemical Study with CMV-Associated Antigen

Aim: To determine the expression of CMV-associated antigen in the human decidual endometrial stromal cells in spontaneous abortions with no evidence of maternal relapse during the first trimester of gestation. Experimental design: We examined 15 placentas resulting from intrauterine fetal death after spontaneous abortion during the 8th, 10th, and 12th week of gestation respectively, and in which CMV reactivation was ruled out from serological evaluation of the pregnant women at admission, versus equal controls after voluntary abortion following well-documented maternal viral recurrence. In addition, a panel of monoclonal antibodies for the identification of leukocytes (CD45/LCA), B-lymphocytes (CD20/L-26), and T-lymphocytes (CD45RO/UCHL1), was performed. All women received hormonal medication to support gestation, in the cases of spontaneous abortions. Results: Immunohistochemical examination using a specific antibody against cytomegalovirus showed large multinucleated infected cells with intranuclear inclusions, located primarily in the decidual stroma within a lymphoplasmacytic infiltrate in the cases of spontaneous abortions. No evidence of infection was observed in the chorionic villi. In the cases of voluntary abortions same findings were observed in the relevant areas, and a strong evidence of infection was observed in the chorionic villi. Conclusion: This study demonstrates 1) that the decidual endometrial stromal cells can express the CMV-associated antigen prior to serological manifestation of the viral replication, 2) the expression of the antigen is higher in cases of hormonal administration to support gestation. In these cases a mild mononuclear infiltrate of UCHL1 (T marker) positive cells, accompanies the CMV-associated antigen positive cells

Does Neoplastic Cholecystokinin Expression Reflect the Embryonal Pattern of the Protein? A Study in Human Pancreas

Aim: To determine the immunoreactivity of cholecystokinin (CCK) during the development of the human fetal pancreas and pancreatic adenocarcinoma, given that, CCK positive cells were demonstrated either in its embryonic anlage or in pancreatic cancer. In order to obtain possible parallels in the expression pattern of neoplastic cells in adults (well – moderately – poorly differentiated), we investigated the pattern of CCK expression in the pancreatic tissue during the various stages of development and compared these with the proliferation of tissue assessed by proliferating cell nuclear antigen (PCNA) immunohistochemistry. Experimental design: Tissue sections from 15 pancreatic fetal specimens, and equal number of ductal adenocarcinoma specimens, were assessed using immunohistochemical methods for CCK. Results: The density of positive cells in the primitive exocrine ductal walls and outgrowing buds was significantly higher than the relevant density in the neoplastic pancreatic tissue of mixed (ductal – endocrine) and pure ductal type (p1=0.004, p2<0.0005, p3<0.0005 and p4=0.023 respectively). The above values were estimated from 20th to 22nd weeks of gestation. There was no significant difference in the density of positive cells in the islet cell epithelium from 25–30 weeks, and the neoplastic tissue of mixed (p5=0.10) and pure ductal type (p6=0.15). Conclusions: The immunostaining for CCK identifies a subgroup of pancreatic ductal adenocarcinomas with a neuroendocrine component (initially considered as pure ductal tumors), and mixed ductal-endocrine tumors. This pattern of expression in neoplasms recapitulates the normal pattern during the embryonal development of the organ, and may be important for the development of new therapeutic approaches with eventual clinical utility