Comparison of Follicular Helper T-Cell Markers with the Expression of the Follicular Homing Marker CXCR5 in Peripheral T-Cell Lymphomas-A Reappraisal of Follicular Helper T-Cell Lymphomas.

Peripheral T-cell lymphomas (PTCLs) expressing multiple follicular T helper (TFH) cell-related antigens are now classified as TFH lymphomas (TFHL), including angioimmunoblastic, follicular, and not otherwise specified (NOS) types. CXCR5 is the TFH cell-defining chemokine receptor that, together with its ligand CXCL13, plays a critical role in the development of follicles and the positioning of TFH and B cells within follicles. A comprehensive immunomorphologic study was performed to investigate the expression pattern of CXCR5 in a large cohort of nodal PTCLs, particularly those with a TFH cell phenotype, and to compare its expression with six other TFH cell-related antigens.

Mitral and Tricuspid Annular Abnormalities in Hypereosinophilic Syndrome-Insights from the Three-Dimensional Speckle-Tracking Echocardiographic MAGYAR-Path Study.

Background: Hypereosinophilic syndrome (HES) is a peripheral eosinophilia characterized by elevated absolute eosinophil cell count ( 1.500 cells/ L) and consequent tissue and end-organ damage. Our aim was to evaluate the mitral annular (MA) and/or tricuspid annular (TA) parameters of patients with HES and to determine whether there are any changes in these parameters compared to healthy individuals.

Left Atrial Volumetric and Functional Properties in Hemophilia - Insights from a Three-Dimensional Speckle-Tracking Echocardiographic MAGYAR-Path Study.

Introduction: Hemophilia is an inherited disorder due to deficiencies in factor VIII (type A) and factor IX (type B). Abnormalities in myocardial mechanics could be theorized due to hemophilia-associated hypocoagubility and related quantitative and qualitative changes of the blood. The present study aimed a detailed assessment of left atrial (LA) volumetric and functional properties in patients with hemophilia using three-dimensional speckle-tracking echocardiography (3DSTE).

Revealing a Phenotypical Appearance of Ibrutinib Resistance in Patients With Chronic Lymphocytic Leukaemia by Flow Cytometry.

Ibrutinib is widely known as an effective and well-tolerated therapeutical choice of the chronic lymphocytic leukaemia (CLL). However, acquired resistance may occur during the treatment, causing relapse. Early detection of ibrutinib resistance is an important issue, therefore we aimed to find phenotypic markers on CLL cells the expression of which may correlate with the appearance of ibrutinib resistance.

The tricuspid annulus in amyloidosis with cardiac involvement: Detailed analysis from the three-dimensional speckle tracking echocardiographic MAGYAR-Path Study.

Introduction: Amyloidosis is a rare condition due to extracellular deposition of excessive amount of protein in parenchymal tissues including the heart. The present study aimed to test whether cardiac amyloidosis (CA) is associated with morphological and functional abnormalities of the tricuspid annulus (TA). For this aim, the results of patients having CA were compared to age- and gender-matched healthy controls by three-dimensional speckle-tracking echocardiography (3DSTE).

Left ventricular rotational abnormalities in hemophilia-insights from the three-dimensional speckle-tracking echocardiographic MAGYAR-Path Study.

Background: Hemophilia is an X-linked inherited disorder primarily affecting males, its major types are type A (deficiency in factor VIII) and B (deficiency in factor IX), and is considered to be the most common severe congenital coagulation factor deficiency. The present study was designed to test whether any differences in left ventricular (LV) rotational mechanics could be demonstrated between male patients with hemophilia and healthy controls using three-dimensional speckle-tracking echocardiography (3DSTE)-derived virtual LV models.

Methods: The present study consisted of 17 patients with hemophilia, however, 3 patients were excluded due to insufficient image quality.

COVID-19 infection in adult patients with hematological malignancies: a European Hematology Association Survey (EPICOVIDEHA).

Background: Patients with hematological malignancies (HM) are at high risk of mortality from SARS-CoV-2 disease 2019 (COVID-19). A better understanding of risk factors for adverse outcomes may improve clinical management in these patients. We therefore studied baseline characteristics of HM patients developing COVID-19 and analyzed predictors of mortality.

Beneficial Effect of Lenalidomide-Dexamethason Treatment in Relapsed/Refractory Multiple Myeloma Patients: Results of Real-Life Data From 11 Hungarian Centers.

In Hungary, the cost of lenalidomide-based therapy is covered only for relapsed multiple myeloma (MM) patients, therefore lenalidomide is typically used in the second-line either as part of a triplet with proteasome inhibitors or as a doublet. Lenalidomide-dexamethasone is a standard treatment approach for relapsed/refractory MM, and according to recent large randomized clinical trials (RCT, the standard arm of POLLUX, ASPIRE, TOURMALINE), the progression-free survival (PFS) is expected to be approximately 18 months. We surveyed ten Hungarian centers treating MM and collected data of 278 patients treated predominantly after 2016.

[Left ventricular deformation in cardiac light-chain amyloidosis and hypereosinophilic syndrome. ].

Hypereosinophilic syndrome (HES) and immunoglobulin light-chain amyloidosis (ALA) are two, rare haematological disorders associated with cardiac alterations. The goal of the present study was a comparative assessment of left ventricular (LV) deformational parameters in HES and ALA patients using three-dimensional speckle-tracking echocardiography (3DSTE). In the present study, results of 10 HES patients (mean age: 60.

Left ventricular rotational mechanics in hypereosinophilic syndrome-Analysis from the three-dimensional speckle-tracking echocardiographic MAGYAR-Path Study.

Introduction: Hypereosinophilic syndrome (HES) is a very heterogeneous group of disorders with varied etiologies characterized by peripheral eosinophilia and eosinophilic tissue/end-organ damage. Three-dimensional speckle-tracking echocardiography (3DSTE) was used for assessment of left ventricular (LV) rotational mechanics in HES patients.

Methods: The study comprised 13 HES patients, from which one patient was excluded due to insufficient image quality.

Real-world data on the efficacy and safety of daratumumab treatment in Hungarian relapsed/refractory multiple myeloma patients.

Daratumumab is a human anti-CD38 monoclonal antibody used in the treatment of refractory and relapsed multiple myeloma. We investigated the efficacy and safety of daratumumab therapy in a real-world setting. Ninety-nine Hungarian patients were included; 48 received monotherapy, while lenalidomide and bortezomib combinations were administered in 29 and 19 cases, respectively.

Right Atrial Deformation Analysis in Cardiac Amyloidosis - Results from the Three-Dimensional Speckle-Tracking Echocardiographic MAGYAR-Path Study.

Background: Light-chain (AL) cardiac amyloidosis (CA) is characterized by fibril deposits, which are composed of monoclonal immunoglobulin light chains. The right ventricle is mostly involved in AL-CA and impairment of its function is a predictor of worse prognosis.

Objectives: To characterize the volumetric and functional properties of the right atrium (RA) in AL-CA by three-dimensional speckle-tracking echocardiography (3DSTE).

Aortic stiffness is increased in patients with hypereosinophilic syndrome being in early necrotic phase.

Background: Persistent eosinophilia and eosinophil-mediated single- or multiple-organ damage are typical features of hypereosinophilic syndrome (HES). Theoretically, eosinophilic infiltration of the ascending aortic wall could not be excluded in HES, therefore the present study aimed to test whether HES is associated with abnormalities in aortic elastic properties.

Methods: The present study comprised 10 HES patients (mean age: 57.

Quantitative assessment of JAK2 V617F and CALR mutations in Philadelphia negative myeloproliferative neoplasms.

Background: Philadelphia negative myeloproliferative neoplasms (MPNs) are characterized by frequent mutations of driver genes including JAK2, CALR and MPL. While the influence of JAK2 V617F mutant allele burden on the clinical phenotype of MPN patients is well-described, the impact of CALR mutant allele burden on clinical features needs further investigation.

Patients And Methods: Quantitative assessment of JAK2 and CALR mutations was performed on diagnostic DNA samples from 425 essential thrombocythemia (ET) and 227 primary myelofibrosis patients using real-time quantitative PCR and fragment length analysis.

Left atrial dysfunction in light-chain cardiac amyloidosis and hypertrophic cardiomyopathy - A comparative three-dimensional speckle-tracking echocardiographic analysis from the MAGYAR-Path Study.

Introduction: While cardiac amyloidosis (CA) is a rare systemic disease characterized by extracellular deposition of protein-derived fibrils, hypertrophic cardiomyopathy (HCM) is histopathologically characterized by myocyte hypertrophy and disarray, interstitial fibrosis, and small intramural coronary arteriole dysplasia. The aim of the present study was to compare left atrial (LA) volumetric and functional characteristics between light-chain (AL) CA and HCM by three-dimensional (3D) speckle-tracking echocardiography (STE).

Methods: The AL-CA group initially consisted of 17 patients with AL-CA, but one patient was excluded due to inadequate image quality, and so the study population consisted of 16 patients (mean age: 64.

[What should we know about cardiac amyloidosis? From clinical signs to treatment].

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.

Cardiac Amyloidosis Associated with Enlargement and Functional Impairment of the Mitral Annulus: Insights from the Three-Dimensional Speckle Tracking Echocardiographic MAGYAR-Path Study.

Background And Aim Of The Study: Cardiac amyloidosis (CA) is a rare condition characterized by the extracellular deposition of amyloidogenic proteins in the heart. The aim of the present study was to compare the size and function of the mitral annulus (MA) between CA patients and age- and gender-matched controls, using three-dimensional speckle-tracking echocardiography (3-D STE).

Methods: The study included 17 patients (mean age 64.

Cardiac amyloidosis is associated with increased aortic stiffness.

Objective: Cardiac amyloidosis (CA) is as an infiltrative disorder primarily caused by extracellular tissue deposition of amyloid fibrils in the myocardial interstitium. The current study was designed to test whether alterations in ascending aortic elastic properties could be detected by echocardiography in CA patients, and to compare their results to controls.

Patients And Methods: We included 19 CA patients from which CA proved to be AL amyloidosis in 17 cases and transthyretin (TTR) amyloidosis in 2 cases.

[State of the art molecular diagnostics and therapy of chronic lymphocytic leukaemia in the era of new targeted therapies].

Chronic lymphoid leukaemia (CLL) has a heterogeneous clinical course depending on many clinical and molecular prognostic markers, which play an important role in the selection of the best treatment option. So far, TP53 disruption is the key prognostic and predictive factor assisting treatment decisions, especially in the era of novel therapies. Asymptomatic patients in early stages of the disease will still benefit from watchful waiting.

[Incidence and treatment of extranodal natural killer/T-cell lymphoma nasal type. Hungarian experiences].

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.