Visszatérő szomatikus mutáció hajas sejtes leukémiában

A hajas sejtes leukémia olyan érett B-sejtes non-Hodgkin-lymphoma, amelyet egyedi klinikai, morfológiai és immunfenotípusbeli sajátosságok jellemeznek. A betegséget splenomegalia, progresszív pancytopenia és relatív indolens lefolyás kíséri. A diagnózis megszületése után a klinikai stádiumtól függ, hogy a „watchful waiting” stratégiát választjuk, avagy azonnali kezelés szükséges. Az első vonalban javasolt purin-nukleozid analógokkal (cladribin, pentostatin) akár több évtizedig tartó komplett remisszió is elérhető. A hajas sejtes leukémia néha igen komoly differenciáldiagnosztikai kérdéseket felvető betegség. A pontos diagnózis igen nagy horderejű, hiszen a rokon kórképek prognózisa és kezelése nagymértékben különbözik a hajas sejtes leukémiáétól. Ezért volt kiemelkedő fontosságú az a felismerés, hogy létezik olyan genetikai elváltozás, amely e betegségek elkülönítő diagnózisában döntő értékű. A BRAF gén V600E szomatikus mutációja hajas sejtes leukémiában szenvedő betegek csontvelői mintáiból izolált DNS-ben minden esetben jelen van, míg a rokon kórképekre ezen mutáció hiánya a jellemző. A szerzők a mutáció felismerésének és a mutációvizsgálat alkalmazásának jelentőségét foglalják össze. Orv. Hetil., 2013, 154, 123–127. | Hairy cell leukemia is a mature B-cell non-Hogkin lymphoma characterized by unique clinical, morphological and immunhistochemical features. Patients with hairy cell leukemia usually present with splenomegaly, progressive pancytopenia and a relative indolent clinical course. The diagnosis does not always indicate immediate treatment, as treatment depends on the clinical stage of the leukemia. Asymptomatic disease without progression requires a watchful waiting policy, while other categories usually need treatment. The treatment of choice is purin nucleosid analogues (pentostatin, cladribine) which can achieve complete remission even for decades. Interferon and monoclonal CD20 antibodies can also significantly prolong tevent free survival. Unfortunately, only the latter two therapies are easily available in Hungary. Splenectomy, which was suggested as first line treatment before the era of purin nucleosid analogues, is only recommended as ultimum refugium. Although hairy cell leukemia is a well-defined lymphoproliferative disease, sometimes it is difficult to differentiate it from other similar entities such as hairy cell leukema variant, splenic marginal zone lymphoma, small lymphocytic lymphoma etc. Making the correct diagnosis is of utmost importance because of the great difference in treatment modalities. Recently, a somatic mutation was found in all analysed hairy cell leukemia samples, but not in other splenic B-cell lymphomas. This article reviews the significance of this observation and presents the different types of methods for the detection of this mutation. Orv. Hetil., 2013, 154, 123–127

The connection between extracurricular, leisure time activities, religiosity and the reasons for drop-out

The interruption of tertiary education and the reduction in the dropout rate have been a central issue in educational sociology and education research. Exploring the possible reasons for dropping out can significantly contribute to reducing the trend. Our aim is to map the links between students dropping out and individual factors. Consequently, we investigate the connection between extracurricular and leisure-time activities, health behaviour and religiosity in relation to dropout. This is explained by the fact that one of the axioms of the literature on dropout is that belonging to civil networks usually strengthens the commitment to the successful completion of studies. In our analysis, we used the database created during the research carried out in 2018 by the Center for Higher Education Research and Development (CHERD-H) in the framework of project No. 123847 of the National Research, Development and Innovation Fund of Hungary, entitled The Role of Social and Organisational Factors in Student Dropout (DEPART 2018, N=605). Our results show that the neglect of study obligations among those who are disappointed in the course and further education is closely related to the shift in value preferences and an increase in the time spent with entertainment activities and partying. It can also be stated that students take part indifferent types of extracurricular activities only to a limited extent, and the different forms of participation in activities and religiosity are not related to the causes of dropout

A Comprehensive Immunophenotypic Marker Analysis of Hairy Cell Leukemia in Paraffin-Embedded Bone Marrow Trephine Biopsies-A Tissue Microarray Study

Hairy cell leukemia (HCL) is an uncommon B cell lymphoproliferation characterized by a unique immunophenotype. Due to low number of circulating neoplastic cells and 'dry tap' aspiration, the diagnosis is often based on BM trephine biopsy. We have performed a consecutive immunohistochemical analysis to evaluate diagnostic usefulness of various HCL markers (CD11c, CD25, CD68, CD103, CD123, CD200, annexin A1, cyclin D1, DBA.44, HBME-1, phospho-ERK1/2, TRAP, and T-bet) currently available against fixation resistant epitopes. We analyzed tissue microarrays consisting of samples gained from 73 small B-cell lymphoma cases, including hairy cell leukemia (HCL) (n = 32), HCL variant (HCL-v) (n = 4), B-cell chronic lymphocytic leukemia (B-CLL) (n = 11), lymphoplasmacytic lymphoma (LPL) (n = 3), mantle cell lymphoma (MCL) (n = 10), splenic diffuse red pulp small B cell lymphoma (SDRPL) (n = 2), splenic B cell marginal zone lymphoma (SMZL) (n = 8), and splenic B cell lymphoma/leukemia, unclassifiable (SBCL) (n = 3) cases. The HCL cases were 100 % positive for all but 2 (DBA.44 and CD123) of these markers. Annexin A1 showed 100 % specificity and accuracy, which was followed by CD123, pERK, CD103, HBME-1, CD11c, CD25, CD68, cyclin D1, CD200, T-bet, DBA.44, and TRAP, in decreasing order. In conclusion, our results reassured the high specificity of annexin A1 and pERK, as well as the diagnostic value of standard HCL markers of CD11c, CD25, CD103, and CD123 also in paraffin-embedded BM samples. Additional markers, including HBME-1, cyclin D1, CD200, and T-bet also represent valuable tools in the differential diagnosis of HCL and its mimics

Monoclonal antibody HBME-1 reacts with a minor subset of B cells with villous surface and can be useful in the diagnosis of hairy cell leukemia and other indolent lymphoproliferations of villous B lymphocytes

The Hector Battifora mesothelial epitope-1 (HBME-1) monoclonal antibody has been generated against human mesothelioma cells and recognizes a biochemically unknown membrane epitope. We have accidentally found that the HBME-1 reacts with scattered lymphocytes showing villous surface in hyperplastic lymphoid tissue. To evaluate its reactivity pattern, we have performed a consecutive immunohistochemical study in nonneoplastic bone marrow and lymphoid samples (n = 40), as well as in malignant lymphoproliferations (n = 427), including hairy cell leukemia (HCL) (n = 72), HCL variant (HCL-v) (n = 13), splenic diffuse red pulp small B cell lymphoma (SDRPL) (n = 8), splenic B cell marginal zone lymphoma (SMZL) (n = 59), and splenic B cell lymphoma/leukemia, not further classifiable on bone marrow morphology (SBCL) (n = 37) cases. The staining pattern of HBME-1 was compared to DBA.44. HBME-1+ villous lymphocytes were constantly detected in low number in nonneoplastic lymphoid tissues. With multicolor immunofluorescence staining, HBME-1+ lymphocytes showed a CD20+/CD79a+/IgM+ B cell phenotype. In B cell lymphoproliferations of villous lymphocytes, HBME-1 reactivity was demonstrated in 96 % of HCL, 39 % of HCL-v, 50 % of SDRPL, 12 % of SMZL, and 19 % of SBCL cases. Nodal and extranodal marginal zone lymphoma cases were positive in 12 % of the cases. A small minority (4 %) of the other B cell lymphomas and no T cell lymphoma revealed tumor cell reactivity with HBME-1. In conclusion, our study has established that HBME-1 reacts with a minor subset of B lymphocytes and a small proportion of B cell lymphomas, which has not been described previously. We suggest that HBME-1 can be a useful marker in the diagnosis of HCL and other indolent lymphoproliferations of villous B lymphocytes

Brain computer interfaces in computer science and engineering areas: a systematic study

Brain-computer interfaces (BCI) are a channel that implements direct communication between the brain and some external unit. Developments of BCIs can provide new application opportunities in a large number of fields of use. In the development of BCI devices, the development of technology and digital technology represented a big change, as it provided the necessary computing power to implement and run the continuously developing signal processing algorithms that ensure processing and evaluation. The aim of this paper is to provide an overview of BCI research results which were published in the engineering field. In the present study, articles that had a greater impact, where the annual average number of citations is greater than 30, in the BCI field were reviewed and processed in a systematic way, in order to make individual research more comparable. The systematic processing was focused on the aims of application, used device/ dataset, applied data process and achieved best accuracy. This systematic study summarizes the most effective methods used in the BCI processing and highlights the future trends. The results showed an accuracy of 85% thanks to increasingly reliable, accurate and cost-effective signal detection and processing devices, as well as algorithms