Langerhans cell sarcoma with pulmonary manifestation, mediastinum involvement and bronchoesophageal fistula. A rare location and difficulties in histopathological diagnosis

Langerhans cell sarcoma, a neoplastic proliferation of Langerhans cells with malignant cytologic features, is a very rare disease. Only a few cases have been documented in the English-language literature. Special methods, like immunohistochemistry and/or ultrastructural examination, are indispensable for appropriate diagnosis. Correct diagnosis is difficult. In fact, the disease is often misdiagnosed. We present the case of a 47 year-old man with a large mass in the middle lobe of the lung, infiltrating anterior mediastinum, with multiple pulmonary round lesions and enlargement of local lymph nodes, and with bronchoesophageal fistula. Clinical examination indicated the possibility of advanced primary lung cancer. However, the first histological diagnosis was Langerhans cell histiocytosis. In spite of treatment, the progression of pulmonary lesions was observed. Therefore, upper- and middle-lobectomy was performed. The diagnosis of Langerhans histiocytosis was confirmed microscopically again. Nevertheless, the patient’s condition deteriorated progressively and he was admitted to the National Tuberculosis and Lung Diseases Research Institute in order to establish a final diagnosis. Revision of earlier resected specimens, as well as an immunohistochemical and ultrastructural examination of samples, taken once again from a bronchial tumor, led to the establishment of a diagnosis of a unique form of Langerhans cell sarcoma with rare pulmonary manifestation.Mięsak z komórek Langerhansa jest niezwykle rzadkim złośliwym rozrostem nowotworowym. Dotychczas w piśmiennictwie anglojęzycznym opisano nieco ponad dwadzieścia przypadków. Rozpoznanie jest bardzo trudne i wymaga zastosowania diagnostyki immunohistochemicznej i/lub ultrastrukturalnej. Często choroba jest rozpoznawana jako inny proces nowotworowy, a nawet jako rozrost łagodny. W prezentowanej pracy przedstawiono przypadek 47-letniego mężczyzny, u którego stwierdzono nieprawidłową guzowatą masę w okolicy płata środkowego płuca, naciekającą przednie śródpiersie, z rozsianymi, okrągłymi zmianami w płucach, z powiększeniem okolicznych węzłów chłonnych oraz z przetoką oskrzelowo-przełykową. Wyniki badań klinicznych wskazywały na możliwość zaawansowanego raka płuca. W badaniu mikroskopowym wycinków pobranych z drzewa oskrzelowego rozpoznano histiocytozę z komórek Langerhansa. Pomimo wdrożonego leczenia obserwowano progresję choroby. W związku z tym zdecydowano się na operację wycięcia płata górnego i środkowego płuca. Ponownie rozpoznano histiocytozę z komórek Langerhansa. Ze względu na dalszy postęp choroby pacjent został przekazany do Instytutu Gruźlicy i Chorób Płuc. Ocena wcześniejszych preparatów histologicznych wycinków pochodzących z oskrzela, z materiału operacyjnego oraz powtórnie pobranych z oskrzela głównego prawego, poszerzona o szeroki panel przeciwciał immunohistochemicznych i badanie ultrastrukturalne pozwoliła ustalić rozpoznanie rzadkiej postaci mięsakowatej histiocytozy z komórek Langerhansa

Comparison of pulmonary and systemic NO- and PGI_{2}-dependent endothelial function in diabetic mice

Diabetes increases the risk of pulmonary hypertension and is associated with alterations in pulmonary vascular function. Still, it is not clear whether alterations in the phenotype of pulmonary endothelium induced by diabetes are distinct, as compared to peripheral endothelium. In the present work, we characterized differences between diabetic complications in the lung and aorta in db/db mice with advanced diabetes. Male, 20-week-old db/db mice displayed increased HbA1c and glucose concentration compatible with advanced diabetes. Diabetic lungs had signs of mild fibrosis, and pulmonary endothelium displayed significantly ultrastructural changes. In the isolated, perfused lung from db/db mice, filtration coefficient (Kf,c) and contractile response to TXA2 analogue were enhanced, while endothelial NO-dependent modulation of pulmonary response to hypoxic ventilation and cumulative production of NO2− were impaired, with no changes in immunostaining for eNOS expression. In turn, 6-keto-PGF1α release from the isolated lung from db/db mice was increased, as well as immunostaining of thrombomodulin (CD141). In contrast to the lung, NO-dependent, acetylcholine-induced vasodilation, ionophore-stimulated NO2− generation, and production of 6-keto-PGF1α were all impaired in aortic rings from db/db mice. Although eNOS immunostaining was not changed, that of CD141 was clearly lowered. Interestingly, diabetes-induced nitration of proteins in aorta was higher than that in the lungs. In summary, diabetes induced marked ultrastructural changes in pulmonary endothelium that were associated with the increased permeability of pulmonary microcirculation, impaired NO-dependent vascular function, with compensatory increase in PGI2 production, and increased CD141 expression. In contrast, endothelial dysfunction in the aorta was featured by impaired NO-, PGI2-dependent function and diminished CD141 expression

Clinical course and outcome of SARS-CoV-2 infection in multiple sclerosis patients treated with disease-modifying therapies — the Polish experience

Introduction. The aim of this study was to report the course and outcome of SARS-CoV-2 infection in multiple sclerosis (MS) patients treated with disease-modifying therapies (DMTs) in Poland. A major concern for neurologists worldwide is the course and outcome of SARS-CoV-2 infection in patients with MS treated with different DMTs. Although initial studies do not suggest an unfavourable course of infection in this group of patients, the data is limited.Materials and methods. This study included 396 MS patients treated with DMTs and confirmed SARS-CoV-2 infection from 28 Polish MS centres. Information concerning patient demographics, comorbidities, clinical course of MS, current DMT use, as well as symptoms of SARS-CoV-2 infection, need for pharmacotherapy, oxygen therapy, and/or hospitalisation, and short-term outcomes was collected up to 30 January 2021. Additional data about COVID-19 cases in the general population in Poland was obtained from official reports of the Polish Ministry of Health.Results. There were 114 males (28.8%) and 282 females (71.2%). The median age was 39 years (IQR 13). The great majority of patients with MS exhibited relapsing-remitting course (372 patients; 93.9%). The median EDSS was 2 (SD 1.38), and the mean disease duration was 8.95 (IQR 8) years. Most of the MS patients were treated with dimethyl fumarate (164; 41.41%). Other DMTs were less frequently used: interferon beta (82; 20.70%), glatiramer acetate (42; 10.60%), natalizumab (35;8.84%), teriflunomide (25; 6.31%), ocrelizumab (20; 5.05%), fingolimod (16; 4.04), cladribine (5; 1.26%), mitoxantrone (3; 0.76%), ozanimod (3; 0.76%), and alemtuzumab (1; 0.25%). The overall hospitalisation rate due to COVID-19 in the cohort was 6.81% (27 patients). Only one patient (0.3%) died due to SARS-CoV-2 infection, and three (0.76%) patients were treated with mechanical ventilation; 106 (26.8%) patients had at least one comorbid condition. There were no significant differences in the severity of SARS-CoV-2 infection regarding patient age, duration of the disease, degree of disability (EDSS), lymphocyte count, or type of DMT used.Conclusions and clinical implications. Most MS patients included in this study had a favourable course of SARS-CoV-2 infection. The hospitalisation rate and the mortality rate were not higher in the MS cohort compared to the general Polish population. Continued multicentre data collection is needed to increase the understanding of SARS-CoV-2 infection impact on the course of MS in patients treated with DMTs

Arytmogenna kardiomiopatia (dysplazja) prawej komory. Etiologia, objawy, diagnostyka i leczenie

Abstract: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disorder characterised by progressive replacement of right ventricle myocardium by either segmental or diffuse fibro-fatty tissue, often with the involvement of the left ventricular wall and manifestations of arrhythmia. Familial occurrence with autosomal dominant inheritance, variable expression and incomplete penetrance is estimated as 1/5000. The recessive form called Naxos disease has also been reported. The etiology and pathogenesis of ARVC are still unknown. Advanced theories of inflammatory or infective etiology and possible mechanism of myocyte loss through apoptosis and/or transdifferentiation into adipocytes are presented in this article. The patterns of two distinct pathomorphology of ARVC based on the nature of myocardial replacement are also presented. The presentation encompasses the wide spectrum of clinical diagnostic features of the disease, including sudden death in the family and abnormalities in ECG, Echo, MRI, angiography, PES exhibiting morphologic and functional changes, and replacement of myocytes by fibro-fatty tissue observed in endomyocardial biopsy specimens. Special attention is drawn to the subtle clinical manifestations and ECG clues of the disease in children and young people. Treatment modalities including drug therapy, ablation, implantable cardioverter defibrillators, antiarrhythmic surgery or heart transplantation are also presented

Langerhans Cell Sarcoma with Pulmonary Manifestation, Mediastinum Involvement and Bronchoesophageal Fistula. A Rare Location and Difficulties in Histopathological Diagnosis

Langerhans cell sarcoma, a neoplastic proliferation of Langerhans cells with malignant cytologic features, is a very rare disease. Only a few cases have been documented in the English-language literature. Special methods, like immunohistochemistry and/or ultrastructural examination, are indispensable for appropriate diagnosis. Correct diagnosis is difficult. In fact, the disease is often misdiagnosed. We present the case of a 47 year-old man with a large mass in the middle lobe of the lung, infiltrating anterior mediastinum, with multiple pulmonary round lesions and enlargement of local lymph nodes, and with bronchoesophageal fistula. Clinical examination indicated the possibility of advanced primary lung cancer. However, the first histological diagnosis was Langerhans cell histiocytosis. In spite of treatment, the progression of pulmonary lesions was observed. Therefore, upper- and middle-lobectomy was performed. The diagnosis of Langerhans histiocytosis was confirmed microscopically again. Nevertheless, the patient’s condition deteriorated progressively and he was admitted to the National Tuberculosis and Lung Diseases Research Institute in order to establish a final diagnosis. Revision of earlier resected specimens, as well as an immunohistochemical and ultrastructural examination of samples, taken once again from a bronchial tumor, led to the establishment of a diagnosis of a unique form of Langerhans cell sarcoma with rare pulmonary manifestation

Folate Content and Yolk Color of Hen Eggs from Different Farming Systems

This study aimed to compare folate contents in hen eggs from four different farming systems, namely organic, free range, barn, and cage one. Folate retention during egg boiling was studied as well. The contents of individual folate vitamers were determined using the high-performance liquid chromatography method (HPLC), following trienzyme treatment. Folate content in eggs differed significantly (p < 0.05) due to the rearing system, with the highest mean content determined in the eggs from organic farming (113.8 µg/100 g). According to this study, one egg (60 g) may provide 40–86 µg of folates, which corresponds to 10–22% of the recommended daily intake for adults, 400 µg according to the Nutrition Standards for the Polish Population. The predominant folate form found in egg was 5-methyltetrahydrofolate, which showed considerably greater stability under boiling compared to 10-formylfolic acid present in a lower amount. In most eggs tested, the losses in total folate content did not exceed 15%. The color of yolk of the most folate-abundant organic eggs, had the highest value of lightness (L*) and the lowest value of redness (a*). This, however, does not correspond to consumer preferences of intense golden yolk color