Acute lymphoblastic leukemia (ALL) with the presence of the Philadelphia chromosome (Ph+) is one of the most aggressive tumors. Improvement in the prognosis of patients occurred with the introduction of tyrosine kinase inhibitors (TKI), which are currently used in combination with chemotherapy. Such treatment is associated with over 90% chance of achieving complete remission. Next generations of TKI allow achieving deeper and longer responses, which translates into event-free and overall survival. Ponatinib (3 rd generation TKI) is currently registered in the treatment of patients with Ph(+) ALL after intolerance and/or resistance of first and second generation TKI or in patients with T315I mutation of BCR-ABL1 kinase. Work on assessing the efficacy and safety of ponatinib in the first line of therapy is ongoing.
{"title":"Ponatynib w leczeniu ostrej białaczki limfoblastycznej z obecnością chromosomu Filadelfia","authors":"Katarzyna Barbara Dulik, Sebastian Giebel","doi":"10.5603/hem.2019.0034","DOIUrl":"https://doi.org/10.5603/hem.2019.0034","url":null,"abstract":"Acute lymphoblastic leukemia (ALL) with the presence of the Philadelphia chromosome (Ph+) is one of the most aggressive tumors. Improvement in the prognosis of patients occurred with the introduction of tyrosine kinase inhibitors (TKI), which are currently used in combination with chemotherapy. Such treatment is associated with over 90% chance of achieving complete remission. Next generations of TKI allow achieving deeper and longer responses, which translates into event-free and overall survival. Ponatinib (3 rd generation TKI) is currently registered in the treatment of patients with Ph(+) ALL after intolerance and/or resistance of first and second generation TKI or in patients with T315I mutation of BCR-ABL1 kinase. Work on assessing the efficacy and safety of ponatinib in the first line of therapy is ongoing.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2020-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87648072","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}
Acute myeloid leukemia (AML) is a highly heterogeneous disease. Mutation with internal tandem duplication of fms-like tyrosine kinase-3 (FLT3-ITD) is one of the two most common driver mutations in AML, the presence of which is associated with a poor outcome. Midostaurin (Mido) is a multi-targeted tyrosine kinase inhibitor that potently inhibits kinase FLT3, other kinases including platelet-derived growth factor receptors α (PDGFR- α ) and b (PDGFR- β ), tyrosine-protein kinase Src, KIT receptor tyrosine kinase, and vascular endothelial growth factor receptor (VEGFR). Mido is the first targeted therapy to demonstrate improved outcome, and as such it represents a game-changer. The RATIFY study demonstrated significant improvements in overall survival, and in event-free survival, when Mido was added to standard chemotherapy in patients with newly diagnosed FLT3-mutated AML. This paper reviews the current clinical evidence regarding Mido and its use in the induction and treatment of relapsed or refractory disease, and maintenance setting.
{"title":"Rola midostauryny w leczeniu ostrej białaczki szpikowej z towarzyszącą mutacją FLT3","authors":"A. Wierzbowska, Magdalena Czemerska","doi":"10.5603/hem.2019.0032","DOIUrl":"https://doi.org/10.5603/hem.2019.0032","url":null,"abstract":"Acute myeloid leukemia (AML) is a highly heterogeneous disease. Mutation with internal tandem duplication of fms-like tyrosine kinase-3 (FLT3-ITD) is one of the two most common driver mutations in AML, the presence of which is associated with a poor outcome. Midostaurin (Mido) is a multi-targeted tyrosine kinase inhibitor that potently inhibits kinase FLT3, other kinases including platelet-derived growth factor receptors α (PDGFR- α ) and b (PDGFR- β ), tyrosine-protein kinase Src, KIT receptor tyrosine kinase, and vascular endothelial growth factor receptor (VEGFR). Mido is the first targeted therapy to demonstrate improved outcome, and as such it represents a game-changer. The RATIFY study demonstrated significant improvements in overall survival, and in event-free survival, when Mido was added to standard chemotherapy in patients with newly diagnosed FLT3-mutated AML. This paper reviews the current clinical evidence regarding Mido and its use in the induction and treatment of relapsed or refractory disease, and maintenance setting.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2020-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81432273","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}
Treatment of patients with refractory or relapse multiple myeloma (MM) is a major therapeutic challenge. The introduction to the therapy of new active drugs, such as immunomodulating drugs (IMiDs) or proteasome inhibitors contributed to a significant increase in the percentage and quality of the obtained responses and, most importantly, to the prolongation of patients’ life. However, MM is a disease with frequent progressions, and choosing the right treatment strategy is much more difficult with each subsequent relapse. The drugs used in the treatment of such patients include pomalidomide — an IMiD, registered in Poland as the third-line therapy for patients with relapsed and refractory MM. The paper presents a case report of a 66-year-old patient diagnosed with IgG lambda MM, who since January 2016 had received 5 lines of treatment with IMiDs, bortezomib and daratumumab, nevertheless she had not met the criteria of response to treatment. In May 2019, the patient was qualified for further treatment with pomalidomide in combination with low doses of oral dexamethasone (POM-DEX). During the treatment, a significant reduction in the level of the monoclonal IgG lambda protein was observed. After receiving two treatment cycles, the patient met the criteria for partial remission, and after 12 cycles the level of monoclonal protein decreased by 78% compared to the period before treatment. The therapy was quite well tolerated. The only side effects observed during treatment were: worse tolerance of physical effort, weight gain and slight edema of lower limbs. Due to the POM-DEX therapy, the patient achieved a durable response to the treatment, despite the rapid relapses of the disease during the previous lines of treatment.
{"title":"Skuteczność terapii pomalidomidem u pacjenta ze szpiczakiem plazmocytowym opornym na inne leki immunomodulujące","authors":"M. Masternak, M. Morawska","doi":"10.5603/HEM.2020.0043","DOIUrl":"https://doi.org/10.5603/HEM.2020.0043","url":null,"abstract":"Treatment of patients with refractory or relapse multiple myeloma (MM) is a major therapeutic challenge. The introduction to the therapy of new active drugs, such as immunomodulating drugs (IMiDs) or proteasome inhibitors contributed to a significant increase in the percentage and quality of the obtained responses and, most importantly, to the prolongation of patients’ life. However, MM is a disease with frequent progressions, and choosing the right treatment strategy is much more difficult with each subsequent relapse. The drugs used in the treatment of such patients include pomalidomide — an IMiD, registered in Poland as the third-line therapy for patients with relapsed and refractory MM. The paper presents a case report of a 66-year-old patient diagnosed with IgG lambda MM, who since January 2016 had received 5 lines of treatment with IMiDs, bortezomib and daratumumab, nevertheless she had not met the criteria of response to treatment. In May 2019, the patient was qualified for further treatment with pomalidomide in combination with low doses of oral dexamethasone (POM-DEX). During the treatment, a significant reduction in the level of the monoclonal IgG lambda protein was observed. After receiving two treatment cycles, the patient met the criteria for partial remission, and after 12 cycles the level of monoclonal protein decreased by 78% compared to the period before treatment. The therapy was quite well tolerated. The only side effects observed during treatment were: worse tolerance of physical effort, weight gain and slight edema of lower limbs. Due to the POM-DEX therapy, the patient achieved a durable response to the treatment, despite the rapid relapses of the disease during the previous lines of treatment.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2020-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77527498","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}
Myelodysplastic syndromes (MDS) are acquired clonal disorders of the hematopoietic system caused by a mutation in hematopoietic stem cells. Depending on clinical and laboratory parameters, MDS is divided into two subgroups: low-risk MDS (LR-MDS) and high-risk MDS (HR-MDS), which determines a range of therapeutic options. Recently, some progress in prolongation and quality of life has been observed, mainly due to new therapeutic and supportive methods. The article provides an overview of the current diagnostic and therapeutic approach to lower-risk MDS, as well as manifests the most recent registered therapeutic methods. Additionally, the paper reviews supportive treatment and drugs in clinical trials.
{"title":"Postępowanie terapeutyczne w zespołach mielodysplastycznych niższego ryzyka","authors":"K. Wiśniewski, A. Gołoś, J. Góra-Tybor","doi":"10.5603/HEM.2020.0042","DOIUrl":"https://doi.org/10.5603/HEM.2020.0042","url":null,"abstract":"Myelodysplastic syndromes (MDS) are acquired clonal disorders of the hematopoietic system caused by a mutation in hematopoietic stem cells. Depending on clinical and laboratory parameters, MDS is divided into two subgroups: low-risk MDS (LR-MDS) and high-risk MDS (HR-MDS), which determines a range of therapeutic options. Recently, some progress in prolongation and quality of life has been observed, mainly due to new therapeutic and supportive methods. The article provides an overview of the current diagnostic and therapeutic approach to lower-risk MDS, as well as manifests the most recent registered therapeutic methods. Additionally, the paper reviews supportive treatment and drugs in clinical trials.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2020-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74584153","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}
Joanna Zaucha, T. Wróbel, A. Czyż, I. Hus, Ewa Lech-Marańda, W. Jurczak, L. Gil, Sebastian Giebel, W. Jędrzejczak, T. Robak, J. Walewski
In Poland, 800–900 new cases of classical Hodgkin’s lymphoma (cHL) are diagnosed annually. Despite relatively good results of first-line treatment, 20–25% of patients relapse and the chances of their cure become much smaller. The paper presents the consensus of the Polish experts on the management of patients with relapsed and refractory cHL in 2020, taking into account generally accepted international recommendations, approved indications and the Polish reimbursement conditions. The proposed recommendations were voted by all authors. The recommendations that were agreed by the majority are presented in the paper. For each recommendation, the most important information supporting its legitimacy and information justifying the dissenting opinions of the authors of the consensus were quoted.
{"title":"Konsensus ekspertów Polskiej Grupy Badawczej Chłoniaków w zakresie postępowania w nawrotowym lub opornym na leczenie klasycznym chłoniaku Hodgkina w 2020 roku","authors":"Joanna Zaucha, T. Wróbel, A. Czyż, I. Hus, Ewa Lech-Marańda, W. Jurczak, L. Gil, Sebastian Giebel, W. Jędrzejczak, T. Robak, J. Walewski","doi":"10.5603/HEM.A2020.0028","DOIUrl":"https://doi.org/10.5603/HEM.A2020.0028","url":null,"abstract":"In Poland, 800–900 new cases of classical Hodgkin’s lymphoma (cHL) are diagnosed annually. Despite relatively good results of first-line treatment, 20–25% of patients relapse and the chances of their cure become much smaller. The paper presents the consensus of the Polish experts on the management of patients with relapsed and refractory cHL in 2020, taking into account generally accepted international recommendations, approved indications and the Polish reimbursement conditions. The proposed recommendations were voted by all authors. The recommendations that were agreed by the majority are presented in the paper. For each recommendation, the most important information supporting its legitimacy and information justifying the dissenting opinions of the authors of the consensus were quoted.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2020-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83494301","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}
A. Dąbrowska-Iwanicka, Katarzyna Błachnio, Renata Woroniecka, Grzegorz Rymkiewicz, J. Walewski
Chronic lymphocytic leukaemia (CLL) is the commonest B-cell malignancy and leukaemia in western countries. It mainly affects the elderly population with a median age of 72. Overall survival varies from 2 to 15 years and depends on clinical and pathological risk factors like age, clinical stage and the presence of genetic mutations with TP53 aberrations conferring the worst prognosis. Indications for therapy include active and symptomatic disease and advanced clinical stage assessed in the Rai or Binet staging system. CLL therapy has been revolutionized in the last years due to the introduction of novel agents — small molecule inhibitors interfering with signalling pathways. These agents proved to be of greater efficacy than immunochemotherapy in both untreated and relapsed patients and led to the significant improvement in survival of CLL patients. These novel agents have been incorporated into new recommendations for CLL treatment. They have also improved the survival of high-risk CLL patients with TP53 aberrations and complex karyotype although their prognosis is still inferior as compared to other patients’ subgroups. Questions concerning optimal sequencing novel agents and the timing of allogeneic bone marrow transplantation remain still open for high-risk group CLL patients.
{"title":"Leczenie wenetoklaksem młodego pacjenta z przewlekłą białaczką limfocytową z delecją 17p i nawrotem choroby po pierwszej linii leczenia — opis przypadku i przegląd literatury","authors":"A. Dąbrowska-Iwanicka, Katarzyna Błachnio, Renata Woroniecka, Grzegorz Rymkiewicz, J. Walewski","doi":"10.5603/HEM.2020.0044","DOIUrl":"https://doi.org/10.5603/HEM.2020.0044","url":null,"abstract":"Chronic lymphocytic leukaemia (CLL) is the commonest B-cell malignancy and leukaemia in western countries. It mainly affects the elderly population with a median age of 72. Overall survival varies from 2 to 15 years and depends on clinical and pathological risk factors like age, clinical stage and the presence of genetic mutations with TP53 aberrations conferring the worst prognosis. Indications for therapy include active and symptomatic disease and advanced clinical stage assessed in the Rai or Binet staging system. CLL therapy has been revolutionized in the last years due to the introduction of novel agents — small molecule inhibitors interfering with signalling pathways. These agents proved to be of greater efficacy than immunochemotherapy in both untreated and relapsed patients and led to the significant improvement in survival of CLL patients. These novel agents have been incorporated into new recommendations for CLL treatment. They have also improved the survival of high-risk CLL patients with TP53 aberrations and complex karyotype although their prognosis is still inferior as compared to other patients’ subgroups. Questions concerning optimal sequencing novel agents and the timing of allogeneic bone marrow transplantation remain still open for high-risk group CLL patients.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2020-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81035348","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}
E. Patkowska, J. Sawczuk-Chabin, Iwona Solarska, Katarzyna Borg, Ewa Lech-Marańda
The development of targeted therapies in AML patients enabling treatment individualization, such as new FLT3 tyrosine kinase inhibitors, is a promising option for improving treatment outcomes and prolonging patient survival. However, the treatment of patients with a high FLT3-ITD allelic ratio (FLT3-ITD high ) associated with an extremely unfavourable prognosis remains a major clinical problem. The study presents a case of a 20-year-old patient with FLT3-ITD high extramedullary AML at diagnosis. Individualized chemotherapy according to the DA ‘3 + 7’ regimen combined with midostaurin was administered. After the induction treatment, complete remission (CR) was not achieved. After second induction chemotherapy, CR1 was achieved with the presence of residua disease. One cycle of consolidation chemotherapy was then administered, and after myeloablative conditioning allogeneic hematopoietic stem cell transplantation from an unrelated donor was performed. The patient has remained in CR with no residual disease for 18 months.
{"title":"Trudności w leczeniu chorego na ostrą białaczkę szpikową z mutacją FLT3-ITD i wysokim stosunkiem allelicznym — oporność na standardową chemioterapię indukującą w połączeniu z midostauryną","authors":"E. Patkowska, J. Sawczuk-Chabin, Iwona Solarska, Katarzyna Borg, Ewa Lech-Marańda","doi":"10.5603/HEM.2020.0045","DOIUrl":"https://doi.org/10.5603/HEM.2020.0045","url":null,"abstract":"The development of targeted therapies in AML patients enabling treatment individualization, such as new FLT3 tyrosine kinase inhibitors, is a promising option for improving treatment outcomes and prolonging patient survival. However, the treatment of patients with a high FLT3-ITD allelic ratio (FLT3-ITD high ) associated with an extremely unfavourable prognosis remains a major clinical problem. The study presents a case of a 20-year-old patient with FLT3-ITD high extramedullary AML at diagnosis. Individualized chemotherapy according to the DA ‘3 + 7’ regimen combined with midostaurin was administered. After the induction treatment, complete remission (CR) was not achieved. After second induction chemotherapy, CR1 was achieved with the presence of residua disease. One cycle of consolidation chemotherapy was then administered, and after myeloablative conditioning allogeneic hematopoietic stem cell transplantation from an unrelated donor was performed. The patient has remained in CR with no residual disease for 18 months.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2020-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79491454","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}
M. Chełstowska, K. Jamroziak, Ewa Lech-Marańda, K. Warzocha
Hodgkin lymphoma (HL) is a form of lymphoma with good prognosis in the majority of patients, who are cured after first line chemotherapy. However, in about 25–30% of patients, the course of the disease is unfavorable and patients require treatment of subsequent lines. In further treatment, intensive polychemotherapy is used, consolidated with hematopoietic stem cell transplantation (autologous or allogenic). Also new drugs are wanted. Immunotherapy is promising, including antibodies against CD30 — brentuximab vedotin, and antibodies, that affect the PD1/PD-L1 and PD-L2 pathways — nivolumab, pembrolizumab. Determining the optimal management of patients with relapse and refractory HL requires further investigation.
{"title":"Rola brentuksymabu vedotin w leczeniu chorych na opornego/nawrotowego chłoniaka Hodgkina na przykładzie dwóch opisów przypadków","authors":"M. Chełstowska, K. Jamroziak, Ewa Lech-Marańda, K. Warzocha","doi":"10.5603/hem.2019.0024","DOIUrl":"https://doi.org/10.5603/hem.2019.0024","url":null,"abstract":"Hodgkin lymphoma (HL) is a form of lymphoma with good prognosis in the majority of patients, who are cured after first line chemotherapy. However, in about 25–30% of patients, the course of the disease is unfavorable and patients require treatment of subsequent lines. In further treatment, intensive polychemotherapy is used, consolidated with hematopoietic stem cell transplantation (autologous or allogenic). Also new drugs are wanted. Immunotherapy is promising, including antibodies against CD30 — brentuximab vedotin, and antibodies, that affect the PD1/PD-L1 and PD-L2 pathways — nivolumab, pembrolizumab. Determining the optimal management of patients with relapse and refractory HL requires further investigation.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-10-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83912857","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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