Diagnostic and therapeutic quandaries in NK-cell lymphomas - a report of two cases

W niniejszej pracy przedstawiono opis przebiegu klinicznego chłoniaków z komórek naturalnej cytotoksyczności (NK). U pierwszego chorego rozpoznanie chłoniaka NK/T nosowego postawiono na podstawie wyniku badania histopatologicznego zmiany guzowatej u nasady nosa (IIA według klasyfikacji Ann Arbor). Chorego leczono według schematów CN3OP (cyklofosfamid, mitoksantron, winkrystyna, prednizon) oraz IVAC (etopozyd, ifosfamid, cytarabina), ale pomimo początkowej dobrej odpowiedzi na chemioterapię u pacjenta szybko nastąpiła progresja choroby w postaci uogólnionych zmian skórnych i zajęcia płuc. Chory zmarł po 7 miesiącach od momentu postawienia rozpoznania wśród objawów niewydolności oddechowo-krążeniowej. U drugiego chorego podstawą rozpoznania chłoniaka blastycznego z komórek NK był wynik badania histopatologicznego skóry i szpiku (IVB według klasyfikacji Ann Arbor). Chory otrzymywał chemioterapię według schematów COP (cyklofosfamid, winkrystyna, prednizon), CC (cyklofosfamid, kladrybina) oraz 2 kursy złożone z mitoksantronu, cytarabiny i etopozydu. Początkowo uzyskano częściową remisję, jednak szybko nastąpiła progresja choroby w obrębie szpiku i skóry, oporna na chemioterapię. Chory zmarł po 9 miesiącach od momentu postawienia rozpoznania z powodu krwawienia do ośrodkowego układu nerwowego w przebiegu zespołu wykrzepiania wewnątrznaczyniowego. Oba przypadki dokumentują trudności diagnostyczne i terapeutyczne w bardzo rzadko występujących w tej szerokości geograficznej chłoniakach z komórek NK.In the present paper we describe clinical course of natural killer (NK) cell lymphomas. In the first patient, diagnosis of nasal NK/T cell lymphoma was based on histopathologic examination of tumor lesion within the base of nose (Ann Arbor IIA). The patient received chemotherapy according to CN3OP (cyclophosphamide, mitoxantrone, vincristine, prednisone) and IVAC (etoposide, ifosfamide, cytarabine) protocols. His initial response to chemotherapy was good, but rapid disease progression within skin and lungs subsequently occured. The patient died due to cardio-pulmonary insufficiency 7 months after lymphoma diagnosis. In the second patient, diagnosis of blastic NK cell lymphoma was based on histopathologic findings in skin and trephine biopses. The patient was treated according to COP (cyclophosphamide, vincristine, prednisone) and CC (cyclophosphamide, cladribine) protocols and after disease progression he received 2 additional courses of mitoxantrone, etoposide and cytarabine. His initial response to chemotherapy was good, but rapid disease progression within bone marrow and skin occured. The patient zajmowadied due to central nervous system hemorrhage in the course of disseminated intravascular coagulopathy 9 months after lymphoma diagnosis. These two case reports reveal diagnostic and therapeutic difficulties of the very rare NK cell lymphomas

Chest pain: is it always what it seems to be?

Pacjent w wieku 56 lat, po pilnej operacji tętniaka rozwarstwiającego aorty (AAD) (31.03.2017 r.), po udrożnieniu tętnicy biodrowej wspólnej (CIA) z powodu ostrego niedokrwienia kończyny dolnej (marzec, 2017 r.), z 70-procentowym dystalnym zwężeniem gałęzi przedniej zstępującej (LAD) (w angiografii tomografii komputerowej [angio-CT], od roku, dotąd niekwalifikowanym do koronarografii/tomografii emisyjnej pojedynczego fotonu), wydolny krążeniowo-oddechowo, przyjęty z tygodniowym wywiadem stałego bólu w klatce piersiowej, promieniującego do okolicy międzyłopatkowej, lewego barku, niezależnego od wysiłku fizycznego, bez duszności. Kolejne elektrokardiogramy: rytm zatokowy miarowy, zmienny oddechowo q III, nieswoiste zmiany odcinka ST-T w odprowadzeniach I, aVL do –0,5 mm, w odprowadzeniach V4–V6 do –1 mm, bez ewolucji. Wartości troponiny T oznaczanej metodą wysokoczułą i frakcji sercowej kinazy kreatynowej w normie. Nitrogliceryna podawana dożylnie spowodowała tylko zmniejszenie dolegliwości. Angio-CT aorty bez zmian w porównaniu z wcześniejszym badaniem (z 17.10.2017 r.): proteza bez przecieku, stare rozwarstwienie od aorty zstępującej do rozwidlenia, obejmujące CIA. Angio-CT tętnic wieńcowych: istotne długie zwężenie LAD. Bez wskazań do interwencji kardiochirurgicznej. Echokardiografia serca: frakcja wyrzutowa lewej komory 65%, dobry odległy efekt operacji naprawczej AV. Rozpoznano ostry zespół wieńcowy–dławicę piersiową (ACS-UA). Koronarografia: LAD segm. 7 długie zwężenie 80%, izolowane. Aortografia: dobry efekt protezowania aorty wstępującej. Jednoczasowo przezskórna interwencja wieńcowa (PCI)-LAD: segm. 7/IDg (bifurkacja) z implantacją stentu uwalniającego lek. Dolegliwości ustąpiły. Nietypowe bóle w klatce piersiowej u chorego z tętniakiem aorty, nawet po skutecznej operacji, mogą odpowiadać ACS, stanowiąc wskazanie do koronarografii/PCI, po wykluczeniu przyczyn ze strony aorty. Opóźniona koronarografia/PCI/leczenie przeciwpłytkowe w ACS zmniejsza szanse powodzenia leczenia. U opisanego pacjenta stwierdzono dodatni wywiad w kierunku chorób aorty, ujemne markery niedokrwienia mięśnia sercowego, ból promieniujący do typowej dla AAD lokalizacji (do pleców). Pierwszym krokiem była angio-CT w celu wykluczenia kolejnego AAD. Ból w klatce piersiowej nie zawsze jest tym, czym się wydaje. Po wykluczeniu najbardziej oczywistej przyczyny konieczne jest przeprowadzenie diagnostyki różnicowej, ponieważ leczenie terapeutyczne odpowiednie dla jednej choroby lub opóźnienie właściwego leczenia może utrudnić/uniemożliwić leczenie innej.A 56-year-old patient after emergency acute aortic dissection (AAD) surgery (31.03.2017), after common iliac artery (CIA) vascular surgery due to acute right lower limb ischemia (March 2017), with a 70% lesion in distal left anterior descending (LAD) in angiography computed tomography (angio-CT) detected a year ago (not yet qualified for coronarography/single-photon emission computed tomography), hemodynamically stable, presented to hospital due to one-week history of constant, continuous chest pain radiating to the back and left shoulder and independent of physical effort, without dyspnea. Electrocardiography (ECG) in hospital at admission (and a day ago): regular sinus rhythm, respiratory variable q III, non-specific ST-T wave abnormalities in leads I, aVL up to –0.5 mm, in leads V4–V6 up to –1 mm, no evolution. Immobilization, nitroglicerin intravenous. administration — only symptoms reduction. High-sensitivity troponin T and creatine kinase-myocardial bound mass negative. Aortic angio-CT scan: in comparison to the previous study (17.10.2017) without significant changes; no leakage signs within the prosthesis; further dissection of the aortic wall from the level of the descending aorta to the bifurcation and CIA. Coronary angio-CT: significant, long stenosis in proximal LAD. Cardiac surgeon consultation: no indications for intervention. Echocardiography: left ventricular ejection fraction 65%, aortic regurgitation trace, right ventricular systolic function preserved. Diagnosis: acute coronary syndrome–unstable angina (ACS-UA). Coronarography: in LAD segm 7 isolated 80% stenosis. Aortography confirmed good prosthetic effect of the ascending aorta. Simultaneously percutaneous coronary intervention (PCI)-LAD, segm 7/IDg (bifurcation) with drug-eluting stent implantation was performed. The symptoms disappeared. Atypical chest pain in a patient with aortic aneurysm, even after successful cardiac surgery, may suggest an ACS, being an indication for coronary angiography/PCI, after exclusion aortic reasons. Delayed coronarography/coronaroplasty/antiplatelet therapy in ACS reduces the chances of successful treatment. The patient had a history of aortic diseases, negative myocardial ischemia markers, pain radiating to typical for AAD location (back pain). The first step was angio-CT to exclude another AAD. Chest pain is not always what it seems to be. After excluding the most obvious cause, it is necessary to perform differentia diagnostics, because therapeutic approach appropriate for one disease or delay in proper treatment may make it difficult/impossible to treat another one

Monte Carlo simulations of ion channeling in crystals containing dislocations and randomly displaced atoms

N/

Results of Polish Adult Leukemia Study Group (PALG) project assessing TP53 mutations with next-generation sequencing technology in relapsed and refractory chronic lymphocytic leukemia patients — an 18-month update

Indtroduction and methods: In chronic lymphocytic leukemia (CLL), molecular and cytogenetic diagnostics are crucial for the determination of accurate prognosis and treatment choice. Among different genetic aberrations, del(17p13) or TP53 mutations constitute high-risk factors, and early identification of such defects is a high priority for CLL patients. While cytogenetic diagnostics is well-established and accessible for the majority of CLL patients in Poland, molecular diagnostics of TP53 mutations is performed only in a few ERIC-certified centers (eight as of September 2020), and only two of these employ next-generation sequencing (NGS) for routine analysis of TP53 status in CLL patients. Here we report the interim results of a project assessing TP53 mutations with NGS technology in relapsed or refractory CLL patients with confirmed negative del(17p13) status. 249 patients from 32 clinical centers were included in the study. Results: NGS analysis revealed TP53 mutations in 42/249 (17%) patients, half of whom (21/249, 8.5%) had subclonal mutations (VAF ≤10%). These results are in line with published data in relapsed/refractory CLL patients. Conclusions: The results of the project demonstrated the feasibility and accuracy of NGS testing in CLL patients despite several initial logistical and technical obstacles. Our study also proved that, with appropriate funding, CLL patients from any hematological center in Poland can have access to state-of-the-art molecular diagnostic

Nonlinear Control of a Doubly Fed Generator Supplied by a Current Source Inverter

Nowadays, wind turbines based on a doubly fed induction generator (DFIG) are a commonly used solution in the wind industry. The standard converter topology used in these systems is the voltage source inverter (VSI). The use of reverse-blocking insulated gate bipolar transistor (RB-IGBT) in the current source inverter topology (CSI), which is an alternative topology, opens new possibilities of control methods. This paper presents a novel power control system for a DFIG supplied by a CSI. The authors propose to use multi-scalar DFIG state variables. A nonlinear control method realized by feedback linearization was used to control the active and reactive powers of the generator. In the feedback linearization controls, the nonlinear DFIG model was taken into account. In the control system structure, classical proportional–integral controllers were used. The control variables were the output current vector components of the CSI. Such approach was named the “current control”. The proposed control method is characterized by good dynamic properties which, combined with the inverter properties in the rotor circuit, allow to increase the quality of the energy transferred to the grid by the generator. In the simulation tests, the correctness of the decoupling of the active and reactive power control loops, the dynamics of controlled power changes, and the change of the machine operating range resulting from the increase of the rotational speed of the generator shaft were controlled. The simulation studies also evaluated the impact of changes in the value of the passive elements of the system on the operation of the generator system. Characteristic operating states of the generator system were analyzed using computer simulations

Finding Relevant Parameters for the Thin-film Photovoltaic Cells Production Process with the Application of Data Mining Methods

International audienc

Annual Trends in Total Ischemic Time and One-Year Fatalities: The Paradox of STEMI Network Performance Assessment

This study is aimed at assessing trends and relations between total ischemic time, the major quality measure of systemic delay, and case-fatality at the population or patient level in response to growing cardiovascular risk and a constant need to shorten the time to treatment in ST-segment elevation myocardial infarction (STEMI). Data from a prospective nationwide registry of STEMI patients admitted between 2006 and 2013 who were treated with primary percutaneous coronary intervention (PCI) were analyzed. Total ischemic time was calculated as the time from the onset of symptoms to primary PCI and was determined as individual and annual. The primary end-point was one-year, all-cause case-fatality. Among the total 70,093 analyzed patients, temporal trends showed significant decrease in total ischemic time (268 vs. 230 minutes, p < 0.001), a worsening of the risk profile and an increase in one-year case-fatality (7.1% vs. 10.8%, p < 0.001). In the multivariate analysis, longer individual total ischemic time was a risk factor for higher mortality (HR 1.024, 95%CI 1.015–1.034, p < 0.001) and remained significant after adjustment for the year of admission. An inverse relation was observed for the median annual time (HR 0.992, 95%CI 0.989–0.994, p < 0.001). Thus, the observed increasing annual trends in case-fatality cannot directly measure the quality of STEMI network performance