Enikő Bagdi

Regulatory T cells in atopic dermatitis: epidermal dendritic cell clusters may contribute to their local expansion

Background  Regulatory T cells (Tregs) have an essential role in tolerance and immune regulation. However, few and controversial data have been published to date on the role and number of these cells in atopic dermatitis (AD).

Amplification of thymosin beta 10 and AKAP13 genes in metastatic and aggressive papillary thyroid carcinomas

Papillary thyroid carcinoma (PTC) is the most common well-differentiated thyroid cancer. Although the great majority of the cases exhibit an indolent clinical course, some of them develop local invasion with distant metastasis, and a few cases transform into undifferentiated/anaplastic thyroid carcinoma with a rapidly lethal course. To identify gene copy number alterations predictive of metastatic potential or aggressive transformation, array-based comparative genomic hybridization (CGH-array) was performed in 43 PTC cases. Formalin-fixed and paraffin-embedded samples from primary tumours of 16 cases without metastasis, 14 cases with only regional lymph node metastasis, and 13 cases with distant metastasis, recurrence or extrathyroid extension were analysed. The CGH-array and confirmatory quantitative real-time PCR results identified the deletion of the EIF4EBP3 and TRAK2 gene loci, while amplification of thymosin beta 10 (TB10) and Tre-2 oncogene regions were observed as general markers for PTC. Although there have been several studies implicating TB10 as a specific marker based on gene expression data, our study is the first to report on genomic amplification. Although no significant difference could be detected between the good and bad prognosis cases in the A-kinase anchor protein 13 (AKAP13) gene region, it was discriminative markers for metastasis. Amplification in the AKAP13 region was demonstrated in 42.9% and 15.4% of the cases with local or with distant metastasis, respectively, while no amplification was detected in non-metastatic cases. AKAP13 and TB10 regions may represent potential new genomic markers for PTC and cancer progression.

«Rotação corporal rígida» ventricular esquerda num doente com linfoma difuso de células B e envolvimento cardíaco. Um caso de ecocardiografia tridimensional de speckle-tracking – estudo MAGYAR-Path

Secondary myocardial involvement by diffuse large B-cell lymphoma is a rare occurrence. Left ventricular (LV) twist is considered an essential part of LV function. In normal circumstances LV twist results from the movement of two orthogonally oriented muscular bands of a helical myocardial structure with consequent clockwise rotation of the base and counterclockwise rotation of the apex. Three-dimensional (3D) speckle-tracking echocardiography (3DSTE) has been found to be feasible for non-invasive 3D quantification of LV wall motion and rotational mechanics. The present report aimed to assess LV twisting motion in a patient with diffuse large B-cell lymphoma with positron emission tomography/computer tomography-proven cardiac involvement by 3DSTE. During 3DSTE, reduction in some segmental radial, longitudinal, circumferential, area and 3D LV strains were found. Apical and basal LV rotations were found to be in the same counterclockwise direction, confirming near absence of LV twist - so-called rigid body rotation.

Nasalis típusú extranodalis natural killer T-sejtes lymphoma hazai előfordulása és kezelésével szerzett tapasztalatok

INTRODUCTION Extranodal natural killer/T (NK/T) cell lymphoma, nasal type (ENKTL) represents a rare subtype of T-cell lymphomas with aggressive clinical behavior according to WHO 2016 classification. AIM ENKTL has distinctive geographic distribution with higher incidence in Asia and Latin America (10% of all non-Hodgkin lymphoma cases), than in Europe and North America (<1%). ENKTL tipically origins from nasopharynx and upper aerodigestive tract. Anthracycline-based chemotherapy regimens are largely ineffective in the treatment of ENKTL. METHOD Our aims were to evaluate the incidence and treatment strategies of ENKTL patients in Hungarian Haematological Centres between 2003 and 2015. Altogether 20 patients with ENKTL were treated in the 4 haematological hospitals (male:female ratio 12:8, with median 49.5 years of age). RESULTS Ten patients had localized (stage I-II) disease at the time of the diagnosis. Seventeen patients were treated with chemotherapy (11/CHOP, CHOP-like, 2/HyperCVAD, 1/ProMACECytaBom, 1/SMILE, 2/others), which was completed with involved-field radiation therapy (IFRT) (40-46 Gy) in 6 cases were used. After first-line therapy 9 patients achieved complete remission (CR), 3 patients had partial remission (PR), 3 patients had progressive disease (PD), and 2 patients had stable disease (SD). Median follow-up was 32 (3-113) months. Five patients received second-line therapy for progressive or recurrent disease [2/DHAP, 1/VIM, 1/HyperCVAD, 1/ProMACECytaBom]. None of the patients achieved CR after second-line therapy. Two patients have undergone autologous hematopoietic stem cell transplantation (HSCT) after the first CR. CONCLUSION ENKTL treatment is more effective with nonanthracycline-containing regimens. L-asparaginase containing chemotherapy and concurrent or sequential chemo-radiotherapy improves survival and CR rates. Orv Hetil. 2017; 158(41): 1635-1641.Absztrakt: Bevezetes: Az extranodalis nasalis tipusu natural killer/T (NK/T) sejtes lymphoma (ENKTL) a T-sejtes lymphomak egyik ritka agressziv megjelenesű formaja, amely elsősorban sinonasalis es nasopharynx kiindulasu. Bar előfordulasa ritka a fejlett nyugati orszagokban, kezelese a hagyomanyos, agressziv lymphomakban alkalmazott antraciklintartalmu kemoterapiaval csekely hatekonysagu. Celkitűzes: ENKTL-esetek előfordulasa hazai hematologiai centrumok altal gondozott non-Hodgkin-lymphomas betegek kozott. Modszer: A szerzők negy magyarorszagi hematologiai centrumban 2003–2015 kozott kezelt 20 ENKTL-beteg klinikai adatait elemeztek. A betegek kozott 12 ferfi es 8 nő volt, median eletkor 49,5 ev (22–84 ev). Eredmenyek: Tiz esetben a betegseg lokalizalt (I–II. stadiumban) volt a diagnozis idejen. Kemoterapias kezelesben reszesult 17 beteg (11 CHOP, CHOP-szerű, kettő hyper-CVAD, egy ProMACECytaBom, egy SMILE, kettő egyeb), amelyet hat esetben erintett mezős besugarzassal (IFRT) egeszitettek ki (40–46 Gy). Az...

Mit kell tudnunk a cardialis amyloidosisról? A tünettantól a kezelésig

Absztrakt: A szisztemas amyloidosis ritka betegseg, amelyben a sziverintettseg viszonylag gyakran fordul elő es a tulelest jelentősen befolyasolja. Az alapbetegseg es a szervi erintettseg szempontjabol uj diagnosztikus eljarasok segitenek a korai diagnozis felallitasaban es a mihamarabbi kezeles megkezdeseben. Sziverintettseg inkabb a monoklonalis immunglobulin-konnyűlanc (AL-amyloidosis) es a transthyretin formaban fordul elő. AL-amyloidosis eseten a sziverintettseg sulyos kovetkezmenyekhez vezet. A kezdeti sulyossag megiteleseben es a kezelesre adott valasz mertekenek mereseben a szivfunkcioval kapcsolatos biomarker-vizsgalatok segitenek. Amyloidosis eseten az eletkorral nő a sziverintettseg, a prevalencia nem ismert pontosan, de feltetelezhető, hogy tobb eset van valojaban, mint amennyi felismeresre kerul. A szerzők a klinikai tunetek, diagnosztikus eljarasok, kiemelten a kardiologiai vizsgalatok jelentőseget ismertetik. Orv Hetil. 2017; 158(46): 1811–1818.Systemic amyloidosis is a rare disease, in which the heart involvement is rather frequent and determines survival remarkably. Regarding the disease and organ involvement, new diagnostic procedures help to establish the diagnosis and to start the adequate treatment as soon as possible. Cardiac involvement is more likely to be characterised by monoclonal immunglobulin free light chain (AL amyloidosis) type and transthyretin type. In case of AL amyloidosis, heart involvement can lead to serious consequences. Biomarker assessments for cardiac function are important to determine disease severity at the beginning and to measure response to the treatment. In case of amyloidosis, the incidence of the heart involvement grows with age. The prevalence is not known exactly, but probably there are more cases than recognised. The authors present the clinical signs and diagnostic methods, emphasizing the importance of the cardiac examination methods. Orv Hetil. 2017; 158(46): 1811-1818.

Large granular lymphocyte leukemia. A rare disease with personalized treatment options

Bevezetes: A nagy, granulalt lymphocytas leukaemia ritka, legtobbszor kronikus lefolyasu betegseg. Vezető tunete a neutropenia, de okozhat mas immun medialta cytopeniat is. A kezeles megkezdeset a tunetek sulyossaga indokolja. Nincs egyseges kezelesi ajanlas, a valasztasanal a klinikai tuneteket kell figyelembe venni. Celkitűzes: A szerzők sajat tapasztalataik alapjan osszefoglaljak a diagnozis fontos lepeseit es a kezeles lehetőseget, illetve szuksegesseget. Modszer: Osszesen 17 nagy, granulalt lymphocytas leukaemias beteg adatait dolgoztak fel retrospektiv modon. Eredmenyek: A 17 beteg kozul 7 esetben volt szukseg kezelesre, kozuluk 4 esetben indokolta ezt a transzfuziot igenylő anaemia, a tobbi betegnel neutropenia miatt indult kezeles. Negy esetben kortikoszteroid adasat kezdtek, egy esetben ciklosporinnal kiegeszitve. Anti-CD52-, kis dozisu metotrexat- es kombinalt kemoterapia egy-egy betegnel tortent. A kezelesek hatasara 5 betegnel sikerult reszleges valaszt elerni, mig ket beteget szepszis kovetkezteben elveszitettek. Kovetkeztetesek: A diagnozis felallitasat kovetően a betegek kisebb hanyada igenyel kezelest. Az immunszuppressziv kezeles eredmenyes lehet, de a hatas gyakran atmeneti. A legsulyosabb szovődmeny a szepszis, amely neutropenias esetekben tovabbra is jelentős mortalitasi kockazattal jar. Orv. Hetil., 2014, 155(11), 414–419. | Introduction: Large granular lymphocyte leukemia is rare, mainly chronic disease. The most common complication is neutropenia, but other immune-mediated cytopenia may also occur. There are no unified treatment recommendations and initiation of treatment mainly depends on the severity of the symptoms. Aim: The aim of the authors was to analyze the main steps of the diagnosis and the necessity and outcome of treatment in their patients diagnosed with large granular lymphocyte leukaemia. Method: The authors retrospectively analyzed the data of 17 large granular lymphocyte leukemia patients. Results: Of the 17 patients, 7 patients required treatment because of transfusion dependent anemia (4 patients) or neutropenia (3 patients). In 4 patients corticosteroid was given (supplemented with cyclosporine in one patients), while the other patients received anti-CD52 (one patient), low dose methotrexate (one patient) and combined chemotherapy (one patient). Five patients achieved partial response, and two patients died in sepsis. Conclusions: In this cohort only a smaller proportion of patients required therapy. Immunosuppression can be successful, but the effect in most cases was temporary. The most serious complication was sepsis, which is associated with a significant risk of mortality in cases with neutropenia. Orv. Hetil., 2014, 155(11), 414–419.INTRODUCTION Large granular lymphocyte leukemia is rare, mainly chronic disease. The most common complication is neutropenia, but other immune-mediated cytopenia may also occur. There are no unified treatment recommendations and initiation of treatment mainly depends on the severity of the symptoms. AIM The aim of the authors was to analyze the main steps of the diagnosis and the necessity and outcome of treatment in their patients diagnosed with large granular lymphocyte leukaemia. METHOD The authors retrospectively analyzed the data of 17 large granular lymphocyte leukemia patients. RESULTS Of the 17 patients, 7 patients required treatment because of transfusion dependent anemia (4 patients) or neutropenia (3 patients). In 4 patients corticosteroid was given (supplemented with cyclosporine in one patients), while the other patients received anti-CD52 (one patient), low dose methotrexate (one patient) and combined chemotherapy (one patient). Five patients achieved partial response, and two patients died in sepsis. CONCLUSIONS In this cohort only a smaller proportion of patients required therapy. Immunosuppression can be successful, but the effect in most cases was temporary. The most serious complication was sepsis, which is associated with a significant risk of mortality in cases with neutropenia.

Nagy, granulált citoplazmájú lymphocytás leukaemia.Ritka betegség, személyre szabott kezelési lehetőségekkel | Large granular lymphocyte leukemia. A rare disease with personalized treatment options

Bevezetes: A nagy, granulalt lymphocytas leukaemia ritka, legtobbszor kronikus lefolyasu betegseg. Vezető tunete a neutropenia, de okozhat mas immun medialta cytopeniat is. A kezeles megkezdeset a tunetek sulyossaga indokolja. Nincs egyseges kezelesi ajanlas, a valasztasanal a klinikai tuneteket kell figyelembe venni. Celkitűzes: A szerzők sajat tapasztalataik alapjan osszefoglaljak a diagnozis fontos lepeseit es a kezeles lehetőseget, illetve szuksegesseget. Modszer: Osszesen 17 nagy, granulalt lymphocytas leukaemias beteg adatait dolgoztak fel retrospektiv modon. Eredmenyek: A 17 beteg kozul 7 esetben volt szukseg kezelesre, kozuluk 4 esetben indokolta ezt a transzfuziot igenylő anaemia, a tobbi betegnel neutropenia miatt indult kezeles. Negy esetben kortikoszteroid adasat kezdtek, egy esetben ciklosporinnal kiegeszitve. Anti-CD52-, kis dozisu metotrexat- es kombinalt kemoterapia egy-egy betegnel tortent. A kezelesek hatasara 5 betegnel sikerult reszleges valaszt elerni, mig ket beteget szepszis kovetkezteben elveszitettek. Kovetkeztetesek: A diagnozis felallitasat kovetően a betegek kisebb hanyada igenyel kezelest. Az immunszuppressziv kezeles eredmenyes lehet, de a hatas gyakran atmeneti. A legsulyosabb szovődmeny a szepszis, amely neutropenias esetekben tovabbra is jelentős mortalitasi kockazattal jar. Orv. Hetil., 2014, 155(11), 414–419. | Introduction: Large granular lymphocyte leukemia is rare, mainly chronic disease. The most common complication is neutropenia, but other immune-mediated cytopenia may also occur. There are no unified treatment recommendations and initiation of treatment mainly depends on the severity of the symptoms. Aim: The aim of the authors was to analyze the main steps of the diagnosis and the necessity and outcome of treatment in their patients diagnosed with large granular lymphocyte leukaemia. Method: The authors retrospectively analyzed the data of 17 large granular lymphocyte leukemia patients. Results: Of the 17 patients, 7 patients required treatment because of transfusion dependent anemia (4 patients) or neutropenia (3 patients). In 4 patients corticosteroid was given (supplemented with cyclosporine in one patients), while the other patients received anti-CD52 (one patient), low dose methotrexate (one patient) and combined chemotherapy (one patient). Five patients achieved partial response, and two patients died in sepsis. Conclusions: In this cohort only a smaller proportion of patients required therapy. Immunosuppression can be successful, but the effect in most cases was temporary. The most serious complication was sepsis, which is associated with a significant risk of mortality in cases with neutropenia. Orv. Hetil., 2014, 155(11), 414–419.INTRODUCTION Large granular lymphocyte leukemia is rare, mainly chronic disease. The most common complication is neutropenia, but other immune-mediated cytopenia may also occur. There are no unified treatment recommendations and initiation of treatment mainly depends on the severity of the symptoms. AIM The aim of the authors was to analyze the main steps of the diagnosis and the necessity and outcome of treatment in their patients diagnosed with large granular lymphocyte leukaemia. METHOD The authors retrospectively analyzed the data of 17 large granular lymphocyte leukemia patients. RESULTS Of the 17 patients, 7 patients required treatment because of transfusion dependent anemia (4 patients) or neutropenia (3 patients). In 4 patients corticosteroid was given (supplemented with cyclosporine in one patients), while the other patients received anti-CD52 (one patient), low dose methotrexate (one patient) and combined chemotherapy (one patient). Five patients achieved partial response, and two patients died in sepsis. CONCLUSIONS In this cohort only a smaller proportion of patients required therapy. Immunosuppression can be successful, but the effect in most cases was temporary. The most serious complication was sepsis, which is associated with a significant risk of mortality in cases with neutropenia.

[Long-term survival after nasal NK/T cell lymphoma].

The nasal NK/T cell lymphoma is a rare, extranodal non-Hodgkin lymphoma in western civilizations, which has poor prognosis. The Epstein-Barr virus can be detected in tumor cells in nearly all cases. There are no definite treatment guidelines in our days. There is no significant difference in survival between radiotherapy and chemotherapy according to Asian studies. In this case study we show our diagnostic procedures, our treatment options and we present the summary of this illness based on the data found in the literature.

Nazális NK/T-sejtes lymphoma hosszú túlélése@@@Long-term survival of the nasal NK/T cell lymphoma

The nasal NK/T cell lymphoma is a rare, extranodal non-Hodgkin lymphoma in western civilizations, which has poor prognosis. The Epstein-Barr virus can be detected in tumor cells in nearly all cases. There are no definite treatment guidelines in our days. There is no significant difference in survival between radiotherapy and chemotherapy according to Asian studies. In this case study we show our diagnostic procedures, our treatment options and we present the summary of this illness based on the data found in the literature.

Long-term survival of the nasal NK/T cell lymphoma

The nasal NK/T cell lymphoma is a rare, extranodal non-Hodgkin lymphoma in western civilizations, which has poor prognosis. The Epstein-Barr virus can be detected in tumor cells in nearly all cases. There are no definite treatment guidelines in our days. There is no significant difference in survival between radiotherapy and chemotherapy according to Asian studies. In this case study we show our diagnostic procedures, our treatment options and we present the summary of this illness based on the data found in the literature.