Haematologia最新文献

{"title":"Nietypowa lokalizacja złogów amyloidu w szpiczaku plazmocytowym","authors":"J. Szymańczyk, R. Pogłód, M. Kraj, A. Michalska, Monika Prochorec-Sobieszek, Janusz Szymańczyk","doi":"10.5603/HEM.2016.0007","DOIUrl":"https://doi.org/10.5603/HEM.2016.0007","url":null,"abstract":"Amyloid deposits in the lower lip were found in a 68-year-old patient with plasma cell myeloma (PCM) (variant: κ light chain disease), leukopenia and coexisting ischemic heart disease four years since the diagnosis of malignancy. The lesion was surgically removed. Inaccessibility of proteasome inhibitors resulted that the patient was treated mainly with cyclophosphamide and dexamethasone. This systemic treatment resulted in partial remission and stabilization of PCM. During further observation the patient experienced severe exacerbation of his ischemic heart disease, heart infarct and severe heart failure as well as PCM progression. Surgical treatment of amyloid tumor was complicated by impaired lip healing. This case indicates that tumors containing amyloid deposits in unusual localisation are likely to occur in the course of PCM.","PeriodicalId":12837,"journal":{"name":"Haematologia","volume":"35 1","pages":"85-92"},"PeriodicalIF":0.0,"publicationDate":"2016-08-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80937499","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Narodowy Instytut Onkologii i Hematologii im. Marii Skłodowskiej-Curie — być albo nie być?","authors":"K. Warzocha","doi":"10.5603/HEM.2015.0045","DOIUrl":"https://doi.org/10.5603/HEM.2015.0045","url":null,"abstract":"January 1, 2016 was scheduled for being the operational launch date of the new Maria Sklodow- ska-Curie Memorial National — Institute of Oncology and Hematology (NIOH), arising from a merger between the Centre for Oncology — Institute (CO-I) with the Institute of Hematology and Transfusion Medicine (IHT). A Council of Ministers’ draft regulation (KRM-110-101-15) had already been published on this matter by the Government Legislative Centre. Following a public hearing held on 13–20 October 2015, this draft was then passed by the Ministry of Health to the Standing Committee of the Council of Ministers for the purpose of being signed by the Prime Minister. For no given reason whatsoever, this legislative process was however abruptly halted; then Minister of Health, Marian Zembala, having chosen not to inform his decision to either the Management Boards or the joint Director of these merging institutions. According to media reporting, it appears this was an attempt to circumvent legally binding regulations by setting aside real estate belonging to the CO-I at the Gliwice branch, due to the decision taken by the Minister of Health without the consent of the institutes’ joint Director nor Management Boards. This prevented consolidating the merger, because of differences still existing in the merger applications, opinions, documentation, resolutions and most importantly in the merging parties themselves which were then being resolved. As a result of such actions, the long-term efforts in organising and financing the harmonisation of administrative and management procedures of the merger at both institutions has been wasted. The beneficial outcomes of the recovery programme implemented at the CO-I from 2012–2015 may have also been squandered; this having been commissioned by the Ministry of Health and the Supreme Chamber of Control, due to an adverse assessment of the CO-I made in previous years. This confusing and socially divisive decision to the public good will thus arrest the further development of the CO-I and IHT, and indeed threaten their continuing to operate in their existing organisational and legal forms; it will also expose the National Treasury to additional future burdens. The presented study serves to document several years of actions undertaken by the Ministry of Health, CO-I and IHT for establishing NIOH. Perhaps it can help guide decision-makers in the future. Undoubtedly, strengthening the capacity for undertaking research, science, education, diagnostics and treatment in both institutes through their consolidation, will be crucial for their developing vital and complementary areas of medicine, namely oncology, hematology and transfusion medicine. Beyond question, establishing NIOH with a status of the Public Research Institute — directly subordinated to the Ministry of Health — for delivering comprehensive diagnostic and therapeutic services, together with providing a centre of excellence for post-graduate education and reseach will ","PeriodicalId":12837,"journal":{"name":"Haematologia","volume":"134 1","pages":"313-327"},"PeriodicalIF":0.0,"publicationDate":"2016-06-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89206828","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"65 lat Instytutu Hematologii i Transfuzjologii - czterej wybitni ludzie, bez których nie byłoby tej historii","authors":"K. Warzocha","doi":"10.5603/HEM.2016.0001","DOIUrl":"https://doi.org/10.5603/HEM.2016.0001","url":null,"abstract":"","PeriodicalId":12837,"journal":{"name":"Haematologia","volume":"306 1","pages":"1-7"},"PeriodicalIF":0.0,"publicationDate":"2016-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77130530","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Instytut Hematologii i Transfuzjologii - działalność naukowa, lecznicza i diagnostyczna","authors":"Instytut Hematologii i Transfuzjologii","doi":"10.5603/Hem.2016.0002","DOIUrl":"https://doi.org/10.5603/Hem.2016.0002","url":null,"abstract":"","PeriodicalId":12837,"journal":{"name":"Haematologia","volume":"1 1","pages":"8-67"},"PeriodicalIF":0.0,"publicationDate":"2016-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87528896","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Haematological and Immunological Changes in the Blood of African Catfish (Clarias gariepinus, Burchell 1822) Reared Under Different Sex Combinations","authors":"E. Ajani, A. Olanrewaju, O. Kareem","doi":"10.3923/HEMATOL.2016.1.5","DOIUrl":"https://doi.org/10.3923/HEMATOL.2016.1.5","url":null,"abstract":"","PeriodicalId":12837,"journal":{"name":"Haematologia","volume":"37 1","pages":"1-5"},"PeriodicalIF":0.0,"publicationDate":"2016-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73517092","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Badania genetyczne w diagnostyce hemofilii B","authors":"E. Odnoczko, J. Windyga","doi":"10.5603/HEM.2015.0038","DOIUrl":"https://doi.org/10.5603/HEM.2015.0038","url":null,"abstract":"Hemophilia A (HA) is one of the most frequently recognized genetically determined bleeding disorders. At its ground is a congenital lack, deficiency or dysfunction of coagulation factor VIII (FVIII), which leads to significant disturbances of hemostatic system. The severity of bleeding diathesis is determined by the degree of FVIII deficiency in plasma. The diagnosis of HA is mainly based on the results of the coagulation plasma test, including measuring the FVIII coagulation activity. Nowadays, it is believed that diagnosis of HA should be extended to genetic testing, to get to know the causative mutation. Determination of the mutation responsible for HA is important for at least two reasons: 1) enables a preliminary assessment of the risk of FVIII inhibitor development and 2) make possible the clear-cut diagnosis of HA carrier status. This paper presents an algorithm forgenetic testing in patients with HA. On top of that characterizes the molecular techniques used indiagnostic process, and discusses the difficulties associated with the interpretation of the results.","PeriodicalId":12837,"journal":{"name":"Haematologia","volume":"1 1","pages":"264-270"},"PeriodicalIF":0.0,"publicationDate":"2015-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82930184","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Limfocytoza CD4+CD8+ oraz wysięki osierdziowy i opłucnowy u chorego na przewlekłą białaczkę szpikową w fazie przewlekłej leczonego dazatynibem z powodu niepowodzenia terapii imatynibem","authors":"K. Lewandowski, Agata Lehman-Kopydłowska, M. Gniot, A. Czyż, B. Ratajczak, Mieczysław Komarnicki","doi":"10.5603/Hem.2015.0041","DOIUrl":"https://doi.org/10.5603/Hem.2015.0041","url":null,"abstract":"Stosowanie inhibitorow kinaz tyrozynowych u chorych na przewleklą bialaczke szpikową (CML) moze prowadzic do zmian w ukladzie immunologicznym. W pracy przedstawiono przebieg choroby u pacjenta w fazie przewleklej CML leczonego interferonem a, a nastepnie — z powodu utraty wiekszej odpowiedzi cytogenetycznej — imatynibem (IM). Po 4 latach leczenia za pomocą IM, mimo uprzedniego uzyskania wiekszej odpowiedzi molekularnej (MMR), potwierdzono utrate calkowitej odpowiedzi cytogenetycznej (opornośc mutacyjna, M244V BCR/ABL1). Zastosowanie w kolejnej linii leczenia dazatynibu w dawce 100 mg/dobe pozwolilo na uzyskanie glebokiej odpowiedzi molekularnej (DMR). W trakcie pierwszych 9 miesiecy terapii tolerancja leczenia byla dobra. Poźniej pojawily sie objawy ogolne (stany podgorączkowe, nadmierne poty), niezyt blony śluzowej nosa, a we krwi obwodowej — leukocytoza z bezwzgledną limfocytozą CD3+, CD8+, HLA-DR+. Po 2 latach, z powodu nasilenia sie wyzej wymienionych dolegliwości, dawke leku zmniejszono do 80 mg/dobe. Przynioslo to ustąpienie wymienionych dolegliwości oraz normalizacje liczby leuko- i limfocytow we krwi. Zmniejszenie dawki leku doprowadzilo jednak do utraty MMR. Powrot do wyjściowej dawki dazatynibu pozwolil ponownie uzyskac DMR. Poprawie jakości odpowiedzi molekularnej towarzyszyl nawrot dolegliwości ogolnych oraz ponowny wzrost limfocytozy we krwi. Po kilku miesiącach leczenia dazatynibem wystąpily wymagające hospitalizacji objawy wysiekowego zapalenia oplucnej i osierdzia. Po ustabilizowaniu stanu chorego zdecydowano o zastosowaniu nilotynibu. W trakcie 12-miesiecznego okresu obserwacji tolerancja leczenia byla dobra. Stosowanie nilotynibu doprowadzilo do szybkiego ustąpienia objawow ogolnych oraz limfocytozy, a takze ponownego uzyskania DMR.","PeriodicalId":12837,"journal":{"name":"Haematologia","volume":"59 1","pages":"301-307"},"PeriodicalIF":0.0,"publicationDate":"2015-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85651639","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Nowe spojrzenie na nocną napadową hemoglobinurię","authors":"Agnieszka Piekarska, Justyna Spychalska","doi":"10.5603/Hem.2015.0035","DOIUrl":"https://doi.org/10.5603/Hem.2015.0035","url":null,"abstract":"Nocna napadowa hemoglobinuria (PNH) nalezy do tak zwanych chorob sierocych. Jest wywolana nabytym defektem klonalnym krwiotworczej komorki macierzystej. Defekt ten polega na niedoborze lub braku na komorkach potomnych bialek blonowych związanych z kotwicą glikozylofosfatydyloinozytolu, w tym inhibitorow dopelniacza CD55 i CD59, bedącym skutkiem mutacji w genie PIGA. W efekcie u pacjentow dochodzi do ciąglej hemolizy związanej z niekontrolowaną aktywacją dopelniacza. Najczestszą przyczyną chorobowości i śmiertelności w tej chorobie są powiklania zakrzepowo-zatorowe. W Polsce, ze wzgledu na brak mozliwości stosowania przeciwciala hamującego uklad dopelniacza — ekulizumabu (leku zarejestrowanego do leczenia chorych z hemolizą i wysoką aktywnością choroby), postepowanie terapeutyczne sprowadza sie do przetaczania koncentratu krwinek czerwonych, profilaktyki przeciwzakrzepowej i przeszczepiania allogenicznych krwiotworczych komorek macierzystych u osob mających zgodnego dawce. W pracy przedstawiono patomechanizm zlozonych zaburzen obserwowanych w PNH ze szczegolnym uwzglednieniem powiklan zakrzepowo-zatorowych oraz omowiono wspolczesne wytyczne postepowania diagnostycznego i terapeutycznego.","PeriodicalId":12837,"journal":{"name":"Haematologia","volume":"20 1","pages":"278-292"},"PeriodicalIF":0.0,"publicationDate":"2015-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85173398","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Etiologia i patogeneza szpiczaka plazmocytowego","authors":"Elżbieta Iskierka-Jażdżewska, K. Jamroziak","doi":"10.5603/HEM.2015.0037","DOIUrl":"https://doi.org/10.5603/HEM.2015.0037","url":null,"abstract":"Szpiczak plazmocytowy (PCM) jest nowotworowym rozrostem komorek plazmatycznych, w ktorym wystepują charakterystyczne objawy uszkodzenia narządow, wtorne do procesu nowotworowego. Mimo znacznego postepu w terapii chorych PCM wiedza na temat etiopatogenezy tej choroby pozostaje niekompletna. Do najlepiej udokumentowanych czynnikow związanych ze zwiekszonym ryzykiem zachorowania na PCM nalezą wystepowanie gammopatii monoklonalnej o nieokreślonym znaczeniu (MGUS), zaawansowany wiek, plec meska oraz określona grupa etniczna. Rozwoj nowotworu plazmocytowego polega na stopniowej ewolucji od wczesnego i bezobjawowego stadium MGUS, przez objawowy PCM, do koncowej fazy bialaczki plazmocytowej. W obecnym modelu patogenezy PCM zaklada sie istnienie dwoch odrebnych szlakow inicjujących transformacje nowotworową — szlak translokacji z udzialem genow immunoglobulin oraz szlak hiperdiploidii (trisomii). Obok zmian molekularnych w obrebie klonu plazmocytowego kluczową role w patogenezie PCM odgrywają interakcje z komorkami mikrośrodowiska szpiku kostnego. Po transformacji nowotworowej, w dalszym etapie progresji PCM, dochodzi do wtornych zaburzen molekularnych, ktore prowadzą do stopniowego uniezaleznienia sie od mikrośrodowiska szpiku kostnego i zazwyczaj zwiekszają agresywnośc nowotworu. Lepsze poznanie zlozonych mechanizmow patogenetycznych PCM w ostatnich latach budzi nadzieje na poszerzenie mozliwości terapeutycznych i dalszą poprawe rokowania w przyszlości.","PeriodicalId":12837,"journal":{"name":"Haematologia","volume":"465 1","pages":"245-263"},"PeriodicalIF":0.0,"publicationDate":"2015-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79868718","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Wyzwania systemowe stojące przed hematologią onkologiczną w aspekcie starzejącego się społeczeństwa w Polsce","authors":"Jakub Gierczyński, Ewa Lech-Marańda, Małgorzata Gałązka-Sobotka, J. Gryglewicz, J. Didkowska, U. Wojciechowska, K. Warzocha","doi":"10.5603/HEM.2015.0040","DOIUrl":"https://doi.org/10.5603/HEM.2015.0040","url":null,"abstract":"Starzenie sie spoleczenstwa i wzrastająca zapadalnośc na nowotwory hematologiczne jest wyzwaniem dla systemu ochrony zdrowia. Nowotwory hematologiczne stanowily okolo 5% wszystkich zachorowan na nowotwory ogolem w 2012 roku, a polowa chorych miala ponad 65 lat. Nowe standardy leczenia oraz technologie medyczne stwarzają szanse dla chorych, ale ich zastosowanie wymaga wnikliwych badan oraz potwierdzenia uzyteczności w ramach praktyki klinicznej. Warunkami adekwatnego dostepu pacjentow w Polsce do technologii medycznych w ramach aktualnych standardow terapeutycznych są optymalizacja i adekwatne finansowanie systemu opieki zdrowotnej. Wydaje sie, ze hematologia onkologiczna moze byc modelowym przykladem efektywnych rozwiązan systemowych — z pacjentem jako podmiotem tego systemu.","PeriodicalId":12837,"journal":{"name":"Haematologia","volume":"52 1","pages":"293-300"},"PeriodicalIF":0.0,"publicationDate":"2015-12-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89162255","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}