HaematologiaPub Date : 2020-01-01DOI: 10.5603/HEM.2020.0028
Joanna Niesiobędzka-Krężel
{"title":"Remisja wolna od leczenia w praktyce klinicznej: opis grupy 6 chorych na przewlekłą białaczkę szpikową, po zaprzestaniu leczenia nilotynibem — doświadczenie jednego ośrodka","authors":"Joanna Niesiobędzka-Krężel","doi":"10.5603/HEM.2020.0028","DOIUrl":"https://doi.org/10.5603/HEM.2020.0028","url":null,"abstract":"In the last 20 years major goals of therapy of chronic myelogenous leukemia (CML) have changed. This leukemia, which was previously a fatal disease, has indeed become a chronic disease thanks to the use of tyrosine kinase inhibitors (TKIs), with life expectancy close to that in the general population. Excellent treatment effectiveness with the achievement of long-term, stable, deep molecular response was the motivation for attempts to stop the therapy with the intention of maintaining remission (TFR, treatment-free remission). Based on many clinical trials with over 3,000 patients and several years of follow-up, it is known that 40–60% of patients will maintain molecular remission and remain untreated, while the rest of them will require TKI to be restarted. Information on the procedure to be followed appeared in scientific societies’ recommendations and some centers treating CML patients have made attempts to stop the therapy. This work describes a group of six patients in the TFR phase after discontinuation of nilotinib used in the first-line treatment.","PeriodicalId":12837,"journal":{"name":"Haematologia","volume":"13 21","pages":"1-7"},"PeriodicalIF":0.0,"publicationDate":"2020-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"91506508","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}
HaematologiaPub Date : 2020-01-01DOI: 10.5603/HEM.A2020.0010
B. Pula, J. Mańko, Monika Pępek, T. Stoklosa, K. Hałaburda, Ewa Lech-Marańda, I. Hus
{"title":"Wenetoklaks w monoterapii przewlekłej białaczki limfocytowej przed powtórnym przeszczepieniem allogenicznych krwiotwórczych komórek macierzystych","authors":"B. Pula, J. Mańko, Monika Pępek, T. Stoklosa, K. Hałaburda, Ewa Lech-Marańda, I. Hus","doi":"10.5603/HEM.A2020.0010","DOIUrl":"https://doi.org/10.5603/HEM.A2020.0010","url":null,"abstract":"Chronic lymphocytic leukemia (CLL) is the most common diagnosed leukemia in adults. Its clinical course is very heterogenous and only allogeneic hematopoietic stem cell transplantation (allo-HSCT) ensures cure of this disease. Introduction of venetoclax, an antagonist of the antiapoptotic BCL-2 protein, significantly improved patient outcome. Herein we present the use of venetoclax monotherapy as a bridging therapy before second allo-HSCT in a patient with relapsed and refractory CLL.","PeriodicalId":12837,"journal":{"name":"Haematologia","volume":"1 1","pages":"95-100"},"PeriodicalIF":0.0,"publicationDate":"2020-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83039606","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}
HaematologiaPub Date : 2019-12-20DOI: 10.5603/hem.2019.0028
Karolina Piechna, Przemyslaw Juszczynski
{"title":"Perspektywy rozwoju strategii terapeutycznych opartych na TRAIL i jego analogach w hematoonkologii","authors":"Karolina Piechna, Przemyslaw Juszczynski","doi":"10.5603/hem.2019.0028","DOIUrl":"https://doi.org/10.5603/hem.2019.0028","url":null,"abstract":"TRAIL (TNF-related apoptosis inducing ligand), member of the tumor necrosis factor superfamily, is known for its strong antitumor activity in many types of cancer cells, while exhibiting relatively low cytotoxicity to most normal cells. Therapeutic strategies utilizing TRAIL or its analogs usually exhibit high activity in preclinical in vitro and in vivo models. In clinical trials, TRAIL and its analogs were generally well tolerated, but, surprisingly, exhibited low activity. Major limitation of TRAIL-based therapies is intrinsic or secondary resistance to apoptosis induction, mediated by a plethora of diverse mechanisms. However, as at least a fraction of these resistance mechanisms are amenable for pharmacological modulation, they represent rational targets for combination therapies synergizing with TRAIL. Herein, we review the preclinical and clinical trials based on combination of pharmaceutics synergizing with TRAIL or TRAIL analogs and discuss the prospects of development of such strategies.","PeriodicalId":12837,"journal":{"name":"Haematologia","volume":"26 1","pages":"148-160"},"PeriodicalIF":0.0,"publicationDate":"2019-12-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74196479","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}
HaematologiaPub Date : 2019-12-20DOI: 10.5603/hem.2019.0029
G. Helbig, Tomasz Sacha, A. Mital, Marek Hus, Bożena Katarzyna Budziszewska, Krystyna Gałązka, Aneta Szudy-Szczyrek, M. Sobas, M. Zawada, Karolina Chromik
{"title":"Zaawansowana mastocytoza układowa — stanowisko ekspertów dotyczące postępowania diagnostycznego i leczniczego","authors":"G. Helbig, Tomasz Sacha, A. Mital, Marek Hus, Bożena Katarzyna Budziszewska, Krystyna Gałązka, Aneta Szudy-Szczyrek, M. Sobas, M. Zawada, Karolina Chromik","doi":"10.5603/hem.2019.0029","DOIUrl":"https://doi.org/10.5603/hem.2019.0029","url":null,"abstract":"Systemic mastocytosis (SM) is characterized by clonal proliferation of abnormal mast cells and their accumulation in skin and/or other organs. The annual incidence of SM is between 5–10 new cases per million population and it usually occurs above 30 years of age. The diagnostic criteria include the detection of aggregates of mast cells in bone marrow, an increased serum tryptase level, the expression of CD25 on the mast cells and the presence of somatic mutation within the KITD816V . An advanced SM encompasses three variants with which hematologists should be familiar with 1) aggressive SM (ASM), 2) SM with an associated hematological neoplasm (SM-AHN) and 3) mast cell leukemia (MCL). Among them MCL has the worst prognosis with median survival of 2 months. The prognostic factors in SM include both clinical, laboratory and molecular parameters, but the latter are of special interest. The adverse prognosis is associated with the mutations within the genes: SRSF2, RUNX1 and ASXL1 . The therapeutic approach independently from SM variant, should be aimed at avoidance of known triggers of mast cell activation and anti-mediator therapy as needed. Midostaurin with overall response rate of 60% should be the first-line choice when the symptoms of organ dysfunction occurs. The other treatments comprise cladribine and [peg]interferon alpha. The only curative therapy for SM is allogeneic stem cell transplantation. In this manuscript we present the current views on diagnostic and therapeutic approach for patients with this rare entity.","PeriodicalId":12837,"journal":{"name":"Haematologia","volume":"11 1","pages":"113-123"},"PeriodicalIF":0.0,"publicationDate":"2019-12-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87829323","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}
HaematologiaPub Date : 2019-12-20DOI: 10.5603/hem.2019.0026
Anna Hanna Holopa, Nicola Szeja, S. Grosicki
{"title":"Zachowania żywieniowe chorych na nowotwory hematologiczne — prospektywne badanie jednoośrodkowe","authors":"Anna Hanna Holopa, Nicola Szeja, S. Grosicki","doi":"10.5603/hem.2019.0026","DOIUrl":"https://doi.org/10.5603/hem.2019.0026","url":null,"abstract":"Introduction. Oncohaematological diseases are a special group of cancer diseases. An important role in their pathogenesis may play exposure to carcinogenic compounds, including substances present in food, such as polycyclic aromatic hydrocarbons (PAH), heterocyclic aromatic amines (HCA), acrylamide, nitrosamines or ethanol. Aims and assumptions were analysis of eating behaviours leading to the consumption of carcinogenic compounds and assessment of frequency of carcinogenic compounds intake. Material and methods. 201 patients with haematological cancers participated in the study. The survey used authorial questionnaire. Selected eating habits and frequency of carcinogenic compounds intake were examined in the study. The results were developed in the Statistica 12 program. Results. Patients presented different eating habits. 28.3% (N = 34) of the respondents did not read the compositions of the products they buy, regardless of their place of residence. 46.0% (N = 92) of respondents most often consumed fried products. Only 30% of men and women (N = 27) negated frying on the same fat. Most women — 44.3% (N = 39) declared consumption of pork, most men 44.2% (N = 50) of poultry. 58.2% of respondents (N = 117) were characterized by low frequency of PAH, HCA and nitrosamine intake. Only 0.5% (N = 1) of the patients had very low acrylamide intake; 56.2% (N = 113) average. 66.2% (N = 133) of the respondents had low frequency of alcohol consumption. Conclusions. Eating habits leading to the consumption of carcinogens are: not reading labels, frying and pork consumption. Patients with hematological cancers had average frequency of carcinogen intake and low frequency of alcohol consumption.","PeriodicalId":12837,"journal":{"name":"Haematologia","volume":"51 1","pages":"124-134"},"PeriodicalIF":0.0,"publicationDate":"2019-12-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77913096","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}
HaematologiaPub Date : 2019-12-20DOI: 10.5603/hem.2019.0027
Karolina Piechna, Przemyslaw Juszczynski
{"title":"Mechanizmy działania i strategie oporności komórek nowotworowych przeciwko apoptozie indukowanej przez TRAIL","authors":"Karolina Piechna, Przemyslaw Juszczynski","doi":"10.5603/hem.2019.0027","DOIUrl":"https://doi.org/10.5603/hem.2019.0027","url":null,"abstract":"TRAIL (TNF-related apoptosis inducing ligand), a protein belonging to the tumor necrosis factor family, causes apoptosis induction through activation of its cognate death receptors (DR4, DR5). Binding TRAIL leads to induction of extrinsic or, in some cell types, intrinsic apoptosis pathways. Because of the lower death receptors surface expression on non-transformed cells than on cancer cells, DR4 and DR5 ligands attract a lot of attention as potential anti-cancer drugs. Despite exhibiting high activity in preclinical in vitro and in vivo models, in clinical trials TRAIL treatment proved to be inefficient. Lack of apoptotic activity is caused by intrinsic or secondary resistance to TRAIL, that is observed in cancer cells. Herein, we systematically review the resistance mechanisms to death-receptor triggered apoptosis. Identification of such mechanisms in clinical setting might serve as a “negative” biomarker, excluding patients unlikely to benefit from TRAIL-based therapies or present a possibility of pharmacological modulation.","PeriodicalId":12837,"journal":{"name":"Haematologia","volume":"14 1","pages":"135-147"},"PeriodicalIF":0.0,"publicationDate":"2019-12-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73926702","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}
HaematologiaPub Date : 2019-06-17DOI: 10.5603/HEM.A2019.0019
Iwona Solarska, B. Pula, Agnieszka Krzywdzińska, K. Jamroziak
{"title":"Zastosowanie sekwencjonowania następnej generacji do oceny heterogenności klonalnej oraz minimalnej choroby resztkowej u chorych na szpiczaka plazmocytowego","authors":"Iwona Solarska, B. Pula, Agnieszka Krzywdzińska, K. Jamroziak","doi":"10.5603/HEM.A2019.0019","DOIUrl":"https://doi.org/10.5603/HEM.A2019.0019","url":null,"abstract":"Plasma cell myeloma (PCM) is a cancer characterized by proliferation of clonal plasmocytes in the bone marrow or extraosseus organs. Type of molecular events, especially of secondary nature, affects the kinetics of disease progression and its clinical heterogeneity in particular patients. Plasma cell myeloma poses an ideal study model of intraclonal heterogeneity due to the high genetical variety of the tumor clone. The process of intraclonal evolution plays a key role in the cancerous transformation of monoclonal gammapathy of undetermined significance and progression of smouldering multiple myeloma to symptomatic PCM. The existence of various cell subclones affects the efficacy of therapeutic strategies and urges the need of identification of novel risk stratification factors which may allow the personalization and optimization of the therapy. Next generation sequencing is an ideal tool enabling the assessment of clonal PCM evolution. This technique is capable of identifying funding mutations defining the aggressiveness of the cell clone. Additionally, it enables the assessment of minimal residual disease (MRD), which is not achievable with routine diagnostic methods. The results of MRD assessment have so far mainly prognostic significance, however in the near future it is most probable that it will be the basis of therapy personalization. The understanding of how genetic changes contribute to clonal evolution and thereby to resistance of plasma cell myeloma cells, will enable to overcome and prevent the development of refractory disease.","PeriodicalId":12837,"journal":{"name":"Haematologia","volume":"63 1","pages":"75-86"},"PeriodicalIF":0.0,"publicationDate":"2019-06-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"72822587","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}
HaematologiaPub Date : 2019-06-15DOI: 10.5603/HEM.A2019.0018
A. Gola
{"title":"Leucocyte and plasma lipids in chronic granulocytic leukaemia.","authors":"A. Gola","doi":"10.5603/HEM.A2019.0018","DOIUrl":"https://doi.org/10.5603/HEM.A2019.0018","url":null,"abstract":"Rozpoznanie zajecia przewodu pokarmowego w przebiegu amyloidozy stanowi wyzwanie diagnostyczne z powodu niespecyficznego i zroznicowanego obrazu klinicznego. Depozyty amyloidu mogą wplywac na strukture i funkcje kazdego narządu ukladu pokarmowego, glownie wątroby i jelita cienkiego. Najczestsze objawy to zmniejszenie masy ciala, przewlekle biegunki, bole brzucha i krwawienia z przewodu pokarmowego. Rozpoznania dokonuje sie na podstawie badania histopatologicznego z barwieniem czerwienią Kongo i z wykazaniem dwojlomności depozytow amyloidowych w świetle spolaryzowanym. Ocena taka powinna byc wykonana u kazdego chorego z niewyjaśnionymi, przewleklymi objawami ze strony przewodu pokarmowego. Z kolei zajecie wątroby objawia sie jej powiekszeniem oraz wzrostem stezenia fosfatazy zasadowej we krwi. Rokowanie w amyloidozie ukladu pokarmowego zalezy od jej etiologii i stopnia uszkodzenia narządow, pogarszają je zajecie wątroby i niedozywienie.","PeriodicalId":12837,"journal":{"name":"Haematologia","volume":"1 1","pages":"243-7"},"PeriodicalIF":0.0,"publicationDate":"2019-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88317893","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}
HaematologiaPub Date : 2019-01-01DOI: 10.5603/HEM.2019.0015
Katarzyna Wicherska-Pawłowska, Aleksandra Bogucka-Fedorczuk, J. Rybka, T. Wróbel
{"title":"Współwystępowanie nowotworów układów krwiotwórczego i chłonnego u tego samego pacjenta — dwa opisy rzadkich przypadków","authors":"Katarzyna Wicherska-Pawłowska, Aleksandra Bogucka-Fedorczuk, J. Rybka, T. Wróbel","doi":"10.5603/HEM.2019.0015","DOIUrl":"https://doi.org/10.5603/HEM.2019.0015","url":null,"abstract":"The coexistence of myeloid and lymphoid malignancies in the same patient is a rare condition. In this article we describe two case reports of patients who developed these two hematological malignancies. The first patient was 25 years-old male, who developed chronic myeloid leukemia 12 months after Burkitt’s lymphoma treatment. In the second patient, 55 years-old female, plasma cell myeloma and polycythemia vera was diagnosed simultaneously. It is still unclear what mechanisms lead to development of two hematological neoplasms originating from two separate cell lineages of hematopoiesis in one patient. Larger studies to establish pathogenesis, epidemiology, prognosis but primarily diagnosis and treatment methods are required.","PeriodicalId":12837,"journal":{"name":"Haematologia","volume":"5 1","pages":"62-68"},"PeriodicalIF":0.0,"publicationDate":"2019-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82514744","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}
HaematologiaPub Date : 2019-01-01DOI: 10.5603/HEM.2019.0013
K. Sokół, Monika Prochorec-Sobieszek, A. Szumera-Ciećkiewicz
{"title":"Znaczenie neprylizyny (CD10) w hematoonkologii","authors":"K. Sokół, Monika Prochorec-Sobieszek, A. Szumera-Ciećkiewicz","doi":"10.5603/HEM.2019.0013","DOIUrl":"https://doi.org/10.5603/HEM.2019.0013","url":null,"abstract":"CD10 is an extracellular protein with enzymatic activity. Its substrates are various substances with the properties of signal particles. CD10 also has in itself the propriety of transmitting signals via the membrane, and affecting various intracellular signal transduction pathways, through interaction with numerous protein complexes. CD10 expression is observed in many normal tissues as well as in selected tumors. It is therefore particularly important in histopathological differential diagnosis. The following article presents the structure, location and functions of the CD10. The effect of CD10 expression on the clinical course and prognosis in hematological and non-haematological malignancies is also described.","PeriodicalId":12837,"journal":{"name":"Haematologia","volume":"5 1","pages":"19-28"},"PeriodicalIF":0.0,"publicationDate":"2019-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74037788","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}