HematologiaPub Date : 2018-10-17DOI: 10.5603/HEM.2018.0025
Agnieszka Krzywdzińska, Iwona Solarska, B. Pula, K. Jamroziak
{"title":"Znaczenie oceny minimalnej choroby resztkowej w amyloidozie AL","authors":"Agnieszka Krzywdzińska, Iwona Solarska, B. Pula, K. Jamroziak","doi":"10.5603/HEM.2018.0025","DOIUrl":"https://doi.org/10.5603/HEM.2018.0025","url":null,"abstract":"Light chain amyloidosis (AL amyloidosis) is a systemic disease leading to organ damage due to deposition of amyloid fibers arising from accumulation of amyloid precursors – monoclonal immunoglobulin light chains produced by clonal bone marrow plasmocytes. The aim of AL amyloidosis therapy is the inhibition amyloidogenic immunoglobulin light chains, what should result in stabilization or often even improvement of involved organs’ function. Treatment effectiveness evaluation is based on hematological and organ response. Despite achievement of complete hematological remission, in some of the patients even a trace number of plasmocytes persisting following treatment may lead to production of low amount of monoclonal immunoglobulins capable of deepening organ damage. These plasmocytes remain undetectable to routine diagnostic approaches and are regarded as minimal residual disease (MRD). Considering the results of the so far published research, it is possible that MRD assessment based on flow cytometric technique may constitute a basic tool of response assessment of AL amyloidosis treatment in the future. In this publication the methodology and results of latest research concerning MRD assessment in AL amyloidosis are presented.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2018-10-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77364949","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}
HematologiaPub Date : 2018-10-17DOI: 10.5603/HEM.2018.0032
Paweł Rubiś, Ewa Dziewięcka, Katarzyna Holcman, Wojciech Szot, Sylwia Wiśniowska-Śmiałek, Agata Leśniak-Sobelga, M. Hlawaty, Piotr Podolec, Magdalena Kostkiewicz
{"title":"Nowe metody diagnostyki amyloidozy serca. Seria przypadków amyloidozy transtyretynowej","authors":"Paweł Rubiś, Ewa Dziewięcka, Katarzyna Holcman, Wojciech Szot, Sylwia Wiśniowska-Śmiałek, Agata Leśniak-Sobelga, M. Hlawaty, Piotr Podolec, Magdalena Kostkiewicz","doi":"10.5603/HEM.2018.0032","DOIUrl":"https://doi.org/10.5603/HEM.2018.0032","url":null,"abstract":"The systemic amyloidosis are diseases induced by misfolded proteins. These insoluble proteins deposit in extracellular space. Infiltration the heart by amyloid can result in progressive diastolic and systolic dysfunction and restrictive cardiomyopathy phenotype – left ventricle hypertrophy and stiffness. More than 20 different precursor proteins have the propensity to form amyloid fibrils. One of the most common amyloid infiltrating the heart is transthyretin amyloid (ATTR) - mostly inherited disease. ATTR is generally considered a mainly neurological disease, but it is phenotypically heterogeneous and the clinical spectrum of the disease varies widely, which makes the diagnosis a real challenge. Although, the early diagnosis improve the prognosis, especially due to new drug introduced in ATTR - tafamidis. In this article we would like to present the case series of transthyretin amyloidosis, which was diagnosed by heart scintigraphy or genetic testing.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2018-10-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80423090","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}
HematologiaPub Date : 2018-10-17DOI: 10.5603/HEM.2018.0030
A. Suska, J. Matyszkiewicz, A. Jurczyszyn
{"title":"Fluktuujący zespół Guillaina-Barré w przebiegu systemowej amyloidozy AL — zwiastun progresji?","authors":"A. Suska, J. Matyszkiewicz, A. Jurczyszyn","doi":"10.5603/HEM.2018.0030","DOIUrl":"https://doi.org/10.5603/HEM.2018.0030","url":null,"abstract":"Primary systemic light-chain amyloidosis (AL) that accounts for 4/5 of all cases can be manifested as a neuropathy. In some cases, neuropathy is a prodromal symptom, present even before the diagnosis or preceding the progression of the disease. Typically, it is symmetrical and progressive, involving the sensitive rather than motor function in the distal parts of the extremities. It can be accompanied by the paresis and autonomic dysfunction. Some atypical presentations of amyloid neuropathy are also mentioned in the literature including asymmetric polyradiculopathy, cranial nerve (III, V, VII) palsies or multiple mononeuropathies. We present a case study of a 58-year-old male patient with AL kappa-light chain amyloidosis of the kidneys, bone marrow and heart, treated in frontline with VD (bortezmib, dexamethasone) and CyBorD (cyclophosphamide, bortezomib, dexamethasone) and with high-dose melphalan followed by autologous hematopoietic stem cell transplantation (auto-HSCT), who developed Guillain-Barre syndrome not responding to standard treatment (intravenous immunoglobulin and plasmapheresis). Severe symptoms of axonal demyelination proceeded the progression of amyloidosis. The improvement in neurological status was obtained by high dose intravenously corticosteroid therapy. Currently, the patient takes dexamethasone as monotherapy – until now without both hematological and neurological progression. Considering the overall clinical picture, the fluctuating Guillain-Barre syndrome may be a prodromal symptom of the amyloidosis progression.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2018-10-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83012286","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}
HematologiaPub Date : 2018-10-17DOI: 10.5603/hem.2018.0020
Redakcja Redakcja „Hematologii”
{"title":"Pytania testowe","authors":"Redakcja Redakcja „Hematologii”","doi":"10.5603/hem.2018.0020","DOIUrl":"https://doi.org/10.5603/hem.2018.0020","url":null,"abstract":"Pytanie 1. Wyniki badań z randomizacją wskazują, że dołączenie kaplacyzumabu do wymiany osocza u chorych na zakrzepową plamicę małopłytkową zwiększa odsetek całkowitych remisji. Działanie tego leku polega na: A. Zwiększeniu aktywności metaloproteinazy ADAMTS13 B. Blokowaniu receptora GPIIb-IIIa na powierzchni płytek krwi C. Degradacji proteolitycznej nieprawidłowych, olbrzymich multimetrów czynnika von Willebranda D. Hamowaniu interakcji między czynnikiem von Willebranda a kompleksem receptorowym GIb–IX–V na płytkach krwi E. Hamowaniu aktywacji dopełniacza","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2018-10-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90362373","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}
HematologiaPub Date : 2018-10-17DOI: 10.5603/Hem.2018.0029
J. Grzybowski, J. Szczygieł, Monika Gawor, P. Michałek, Agnieszka Sioma, Natalia Ojrzyńska, Ł. Mazurkiewicz, Marta Legatowicz-Koprowska, E. Walczak, Maria Franaszczyk, Magdalena Marczak
{"title":"Amyloidoza łańcuchów lekkich immunoglobulin z punktu widzenia kardiologa","authors":"J. Grzybowski, J. Szczygieł, Monika Gawor, P. Michałek, Agnieszka Sioma, Natalia Ojrzyńska, Ł. Mazurkiewicz, Marta Legatowicz-Koprowska, E. Walczak, Maria Franaszczyk, Magdalena Marczak","doi":"10.5603/Hem.2018.0029","DOIUrl":"https://doi.org/10.5603/Hem.2018.0029","url":null,"abstract":"Light-chain amyloidosis (amyloidosis AL) is diagnosed in approx. 70% of patients with cardiac amyloidosis. This type of amyloidosis has the worst prognosis, especially if the diagnosis is made in advanced stages. The majority of patients are referred to a cardiologist, but unfortunately only every fifth of them has the proper diagnosis. Therefore, strategies promoting early diagnosis are important. One of them is the measurement of serum free light chains concentration in every patient with heart failure with preserved ejection fraction. The acknowledgement of free light chains (FLCs) cardiotoxicity rendered the picture of AL amyloidosis from infiltrative cardiomyopathy into a toxic one. Best improvement in regard to heart failure is achieved upon hematological treatment resulting in decrease of FLCs concentration. Therefore, cardiological treatment is rather a supportive therapy. The role of cardiologist is the rapid diagnosis of the disease and referral of the patient to the hematologist. The standard heart failure treatment encompassing use of beta-blockers and angiotensin converting enzyme inhibitors aggravates orthostatic hypotension and congestion. Instead, up-to-date hematological treatment improves the prognosis of AL amyloidosis markedly, as long as early diagnosis is made.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2018-10-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85281713","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}
HematologiaPub Date : 2018-10-17DOI: 10.5603/Hem.2018.0027
Justyna Łyczkowska-Piotrowska, Aleksander Salomon-Perzyński, Agnieszka Końska, K. Jamroziak
{"title":"Doksycyklina w terapii amyloidozy układowej z zajęciem serca","authors":"Justyna Łyczkowska-Piotrowska, Aleksander Salomon-Perzyński, Agnieszka Końska, K. Jamroziak","doi":"10.5603/Hem.2018.0027","DOIUrl":"https://doi.org/10.5603/Hem.2018.0027","url":null,"abstract":"The leading strategy in the management of systemic amyloidosis is currently focused on reducing the production of amyloid precursor proteins. This approach is based on the use of chemotherapy in light chain amyloidosis (AL amyloidosis) or liver transplantation, or attempts to suppress transthyretin gene expression (TTR) in patients with transthyretin amyloidosis (ATTR). Recently, however, therapies have been increasingly developed to reduce the formation of amyloid deposits from circulating precursors or to eliminate already formed amyloid deposits. This approach includes, in particular, the chronic use of doxycycline, a well-known bacteriostatic antibiotic from the tetracycline group. \u0000 In preclinical studies it was shown that the anti-amyloidogenic potential of doxycycline in AL amyloidosis depends on interference in the process of amyloidogenesis and the destruction of amyloid deposits. Clinical retrospective studies indicate that doxycycline used with standard chemotherapy improves prognosis in patients with AL amyloidosis with heart involvement, which is the most unfavorable prognostic group, while maintaining a favorable safety profile of therapy. In contrast, in ATTR, doxycycline appears to stabilize the clinical course of the disease. \u0000 In this paper, we review literature on the role of doxycycline therapy in the treatment of systemic amyloidosis.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2018-10-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86454675","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}
HematologiaPub Date : 2018-08-17DOI: 10.5603/Hem.2018.0017
Agnieszka Ożańska, M. Sobas, Magdalena Olszewska-Szopa, T. Wróbel
{"title":"Nowotwór nerki u pacjentów ze szpiczakiem plazmocytowym — opis dwóch przypadków i przegląd piśmiennictwa","authors":"Agnieszka Ożańska, M. Sobas, Magdalena Olszewska-Szopa, T. Wróbel","doi":"10.5603/Hem.2018.0017","DOIUrl":"https://doi.org/10.5603/Hem.2018.0017","url":null,"abstract":"Multiple myeloma (MM) is characterized by malignant spreading of monoclonal plasma cells in bone marrow. Renal cell carcinoma (RCC) is a result of neoplastic proliferation of epithelial cells in nephron proximal convoluted tubule and forms 95% of malignant kidney’s neoplasms. There are some reports about coexistence of MM and RCC but it is a rare phenomenon. We describe two cases of patients in which during the therapy of MM, RCC was detected accidentally. Radical nephrectomy in early stage of RCC allowed complete recovery and did not interrupt the continuation of MM’s therapy. Additionally, similar cases from literature are discussed.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2018-08-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82097169","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}
HematologiaPub Date : 2018-08-17DOI: 10.5603/HEM.2018.0012
K. Wiśniewski, E. Białopiotrowicz, J. Góra-Tybor
{"title":"Zaburzenia mechanizmów epigenetycznych w ostrej białaczce szpikowej","authors":"K. Wiśniewski, E. Białopiotrowicz, J. Góra-Tybor","doi":"10.5603/HEM.2018.0012","DOIUrl":"https://doi.org/10.5603/HEM.2018.0012","url":null,"abstract":"Epigenetic regulation influences gene expression without changing the nucleotide sequence of the deoxyribonucleic acid (DNA). The most important epigenetic mechanisms include DNA methylation, modifications of histone proteins and non-coding RNAs. The dysregulation of the above mentioned processes plays a significant role in the pathogenesis of acute myeloid leukemia (AML). Mutations in the genes that are essential for epigenetic regulations are common in 70% of patients with AML. The most frequent mutations involve the DNMT3A, TET2, IDH1/2 and ASXL1 genes. Their presence or absence may constitute a vital prognostic factor in the future as well as become a potential basis for targeted therapies. The present paper manifests the importance of epigenetic alterations in the development of acute myeloid leukemia and their impact on the course of the disease. The article also discusses some possibilities for the use of epigenetic modifications in the AML therapy.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2018-08-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79391447","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}
HematologiaPub Date : 2018-08-17DOI: 10.5603/HEM.2018.0014
K. Wiśniewski, J. Góra-Tybor
{"title":"Leki wpływające na mechanizmy epigenetyczne w ostrej białaczce szpikowej","authors":"K. Wiśniewski, J. Góra-Tybor","doi":"10.5603/HEM.2018.0014","DOIUrl":"https://doi.org/10.5603/HEM.2018.0014","url":null,"abstract":"Currently, two DNA methyltransferase inhibitors, azacitadine and decitabine, are the epigenetic agents used for AML treatment. As a result of DNA hipomethylation, these DNA methyltransferase inhibitors contribute to the reactivation of methylation silence tumor suppressor genes. Azacitadine and decitabine can increase life expectancy of older patients precluded from intensive chemotherapy. New studies are being conducted in order to determine the use of azacitadine and decitabine in prevention and treatment of AML relapse after allogeneic hematopoietic stem cell transplantation. \u0000The present paper manifests the mechanism of action of hypomethylating drugs, as well as provides a brief overview of some clinical trials concerning the use of azacitadine and decitabine in AML. The article also discusses some potential epigenetic drugs that are undergoing clinical trials.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2018-08-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79991251","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}
HematologiaPub Date : 2018-08-17DOI: 10.5603/HEM.2018.0018
M. Fejklowicz, S. Grosicki
{"title":"Ostra białaczka z koekspresją antygenów limfo- i mieloidalnych oraz masywną limfadenopatią jako problem diagnostyczny i terapeutyczny","authors":"M. Fejklowicz, S. Grosicki","doi":"10.5603/HEM.2018.0018","DOIUrl":"https://doi.org/10.5603/HEM.2018.0018","url":null,"abstract":"The paper describes the case of a 20-year-old patient with acute leukemia of undetermined linear origin accompanied by massive lymphadenopathy. The patient was treated with acute lymphoblastic leukemia regimen, according to the PALG ALL6 protocol, achieving complete hematological and metabolic remission of the underlying disease.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2018-08-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84840359","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}
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