We present the case of 75 year-old-man who was treated with azacitidine because of myelodysplastic syndrome RAEB-2 (refractory anemia with excess of blasts 2). The treatment was started with azacitidine, erytropoietin and regular blood transfusion (13 units in total). After 4 courses of treatment the blood picture has normalized and the program of regular venesections was implemented. The patient underwent 11 phlebotomy sessions which resulted in ferritin level decrease from 879 μ g/mL to 471 μ g/mL. After 27 cycles of azacitidine treatment the disease transformed to acute myeloblastic leukamia. The patient survived 3 years from the diagnosis of MDS. We believe that signifficant reduction of ferritin level prevented the development of iron overload symptoms and delayed transformation to acute leukemia.
{"title":"Leczenie upustami krwi przeładowania żelazem u pacjenta z zespołem mielodysplastycznym w trakcie skutecznego leczenia azacytydyną","authors":"Hanna Ciepłuch","doi":"10.5603/hem.2019.0002","DOIUrl":"https://doi.org/10.5603/hem.2019.0002","url":null,"abstract":"We present the case of 75 year-old-man who was treated with azacitidine because of myelodysplastic syndrome RAEB-2 (refractory anemia with excess of blasts 2). The treatment was started with azacitidine, erytropoietin and regular blood transfusion (13 units in total). After 4 courses of treatment the blood picture has normalized and the program of regular venesections was implemented. The patient underwent 11 phlebotomy sessions which resulted in ferritin level decrease from 879 μ g/mL to 471 μ g/mL. After 27 cycles of azacitidine treatment the disease transformed to acute myeloblastic leukamia. The patient survived 3 years from the diagnosis of MDS. We believe that signifficant reduction of ferritin level prevented the development of iron overload symptoms and delayed transformation to acute leukemia.","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":"80597744","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. Pluta, Justyna Gil, Jacek Krzanowski, Sławomir Radwański
Progress in Hodgkin lymphoma (HL) treatment has been achieved due to introduction of new diagnostic methods, disease staging, and new therapy implementations. In spite of it, some patients are challenging in diagnostic and therapeutic process. The topic will provide a brief review of 2 young patients with HL who failed to achieve remission following few therapeutic treatment lines. Both of them response has been achieved when brentuximab vedotin was used. Many patients requires up to date new diagnostic methods implementation. Their interpretation is possible only in the context of clinical data.
{"title":"Długotrwałe odpowiedzi na leczenie brentuksymabem vedotin u chorych na chłoniaka Hodgkina","authors":"A. Pluta, Justyna Gil, Jacek Krzanowski, Sławomir Radwański","doi":"10.5603/hem.2019.0020","DOIUrl":"https://doi.org/10.5603/hem.2019.0020","url":null,"abstract":"Progress in Hodgkin lymphoma (HL) treatment has been achieved due to introduction of new diagnostic methods, disease staging, and new therapy implementations. In spite of it, some patients are challenging in diagnostic and therapeutic process. The topic will provide a brief review of 2 young patients with HL who failed to achieve remission following few therapeutic treatment lines. Both of them response has been achieved when brentuximab vedotin was used. Many patients requires up to date new diagnostic methods implementation. Their interpretation is possible only in the context of clinical data.","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":"78933656","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}
Patients with relapsed or primary resistant Hodgkin lymphoma (HL) who do not qualify for high-dose chemotherapy with a transplant of autologous hematopoietic stem cell transplantation (auto-HSCT) have poor prognosis. The use of new drugs such as brentuximab vedotin, anti-PD-L1 antibodies enables remission and sustained response in this difficult to treat group of patients. Brentuximab vedotin is a conjugate of anti-CD30 and auristatin E antibodies. The drug was registered by the U.S. Food and Drug Administration (FDA) and the European Medicines Agency (EMA) in 2012 to treat patients with recurrent or resistant HL. In Poland, this drug is available in the National Health Fund drug program for patients after autologous peripheral blood stem cell transplantation or after at least two lines of chemotherapy, when auto-HSCT is not a valid treatment option. This case illustrates the effectiveness of brentuximab vedotin in the fourth line of primary resistant HL treatment.
{"title":"Skuteczność brentuksymabu vedotin w czwartej linii leczenia chorego na pierwotnie opornego chłoniaka Hodgkina","authors":"Beata Kania-Zembaczyńska","doi":"10.5603/hem.2019.0022","DOIUrl":"https://doi.org/10.5603/hem.2019.0022","url":null,"abstract":"Patients with relapsed or primary resistant Hodgkin lymphoma (HL) who do not qualify for high-dose chemotherapy with a transplant of autologous hematopoietic stem cell transplantation (auto-HSCT) have poor prognosis. The use of new drugs such as brentuximab vedotin, anti-PD-L1 antibodies enables remission and sustained response in this difficult to treat group of patients. Brentuximab vedotin is a conjugate of anti-CD30 and auristatin E antibodies. The drug was registered by the U.S. Food and Drug Administration (FDA) and the European Medicines Agency (EMA) in 2012 to treat patients with recurrent or resistant HL. In Poland, this drug is available in the National Health Fund drug program for patients after autologous peripheral blood stem cell transplantation or after at least two lines of chemotherapy, when auto-HSCT is not a valid treatment option. This case illustrates the effectiveness of brentuximab vedotin in the fourth line of primary resistant HL treatment.","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":"80757936","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}
For elderly patients with myelodysplastic syndrome (MDS) the improvement of the quality of life is one of the most important aspects of the treatment, with the particular role of becoming transfusion independent. Currently, the median age of onset for the MDS in Poland is 71-years-old. In Europe we are observing the constant ageing of the population, which leads to the increase in the incidence of MDS in elderly patients, who are very often encumbered with comorbidities. In the coming 50 years the 4-fold rise of the people above 85 years is expected to be observed, which will be paired with the inceased of MDS cases. This paper describes a patient with MDS treated with azacitidine. After 4 cycles of hypomethylating agents treatment patient became tranfusion independent. Treatment was continued for 19 cycles. After 6 cycles complete remission has been achieved.
{"title":"Wpływ azacytydyny na poprawę jakości życia u pacjentów w podeszłym wieku z zespołami mielodysplastycznymi z grupy pośredniego-2 ryzyka","authors":"Judyta Strzała, W. Baran","doi":"10.5603/hem.2019.0005","DOIUrl":"https://doi.org/10.5603/hem.2019.0005","url":null,"abstract":"For elderly patients with myelodysplastic syndrome (MDS) the improvement of the quality of life is one of the most important aspects of the treatment, with the particular role of becoming transfusion independent. Currently, the median age of onset for the MDS in Poland is 71-years-old. In Europe we are observing the constant ageing of the population, which leads to the increase in the incidence of MDS in elderly patients, who are very often encumbered with comorbidities. In the coming 50 years the 4-fold rise of the people above 85 years is expected to be observed, which will be paired with the inceased of MDS cases. This paper describes a patient with MDS treated with azacitidine. After 4 cycles of hypomethylating agents treatment patient became tranfusion independent. Treatment was continued for 19 cycles. After 6 cycles complete remission has been achieved.","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":"90646558","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}
Azacitidine is a hypomethylating agent, commonly used in treatment of acute myeloid leukemia with 20–30% bone marrow blasts. We present a case of a long-term efficacy of this drug used in the patient for 36 months.
{"title":"Długoterminowa skuteczność azacytydyny u chorej z ostrą białaczką szpikową na podłożu zmian dysplastycznych","authors":"M. Wójtowicz","doi":"10.5603/hem.2019.0004","DOIUrl":"https://doi.org/10.5603/hem.2019.0004","url":null,"abstract":"Azacitidine is a hypomethylating agent, commonly used in treatment of acute myeloid leukemia with 20–30% bone marrow blasts. We present a case of a long-term efficacy of this drug used in the patient for 36 months.","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":"84400546","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}
The standard of care for patients with refractory or relapsed Hodgkin lymphoma is salvage chemotherapy followed by autologous hematopoietic stem cell transplantation. This method is not always effective, however. For patient who do not respond or those who relapse again treatment opportunities are limited. Before of the brentuximab vedotin ”era” the prognosis of such patients was poor, with a 5-year overall survival not exceeding 30%. Allogeneic stem cell transplantation (allo-HSCT) may be curative, but its success is highly dependent on prior good disease control. Brentuximab vedotin can be effective bridging treatment before allo-HSCT because 34% of patients can achive complete remission. In addition, in some patients, used as monotherapy without additional consolidation treatment allows for long-term responses.
{"title":"Długotrwała odpowiedź na leczenie brentuksymabem vedotin u pacjenta z nawrotem chłoniaka Hodgkina po auto-HSCT","authors":"Edyta Subocz, J. Hałka","doi":"10.5603/hem.2019.0023","DOIUrl":"https://doi.org/10.5603/hem.2019.0023","url":null,"abstract":"The standard of care for patients with refractory or relapsed Hodgkin lymphoma is salvage chemotherapy followed by autologous hematopoietic stem cell transplantation. This method is not always effective, however. For patient who do not respond or those who relapse again treatment opportunities are limited. Before of the brentuximab vedotin ”era” the prognosis of such patients was poor, with a 5-year overall survival not exceeding 30%. Allogeneic stem cell transplantation (allo-HSCT) may be curative, but its success is highly dependent on prior good disease control. Brentuximab vedotin can be effective bridging treatment before allo-HSCT because 34% of patients can achive complete remission. In addition, in some patients, used as monotherapy without additional consolidation treatment allows for long-term responses.","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":"88411362","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 syndrome (MDS) is a heterogeneous group of malignant diseases, diverse in terms of clinical course, response to treatment, and cytogenetic and molecular. In the present paper, we reported a case of MDS-RCMD (classified according to WHO 2008); risk intermediate-2, in an 72-years old male patient who was treated 44 cycles azacytidine. After two cycles of AZA, has become independent of blood transfusions, and after six cycles haematological complete response was observed.
{"title":"Długoterminowe leczenie azacytydyną chorego na zespół mielodysplastyczny wyższego ryzyka","authors":"W. Piszczek, Grzegorz Charliński","doi":"10.5603/hem.2019.0001","DOIUrl":"https://doi.org/10.5603/hem.2019.0001","url":null,"abstract":"Myelodysplastic syndrome (MDS) is a heterogeneous group of malignant diseases, diverse in terms of clinical course, response to treatment, and cytogenetic and molecular. In the present paper, we reported a case of MDS-RCMD (classified according to WHO 2008); risk intermediate-2, in an 72-years old male patient who was treated 44 cycles azacytidine. After two cycles of AZA, has become independent of blood transfusions, and after six cycles haematological complete response was observed.","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":"73698585","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}
Agnieszka Pluta, Dorota Jesionek-Kupnicka, E. Wawrzyniak, A. Wierzbowska
{"title":"Leczenie azacytydyną chorego na wtórną do mielofibrozy ostrą białaczkę szpikową","authors":"Agnieszka Pluta, Dorota Jesionek-Kupnicka, E. Wawrzyniak, A. Wierzbowska","doi":"10.5603/hem.2019.0003","DOIUrl":"https://doi.org/10.5603/hem.2019.0003","url":null,"abstract":"","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":"89693886","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}
Anaplastic large cell lymphoma (ALCL) is rare hematological malignancy and subtype of mature T-cell lymphoma. Most patients with diagnosed systemic ALCL (sALCL) respond to common first-line chemotherapy based on antracycline (CHOP [doxorubicin, vincristine, prednisone], or CHOEP [doxorubicin, vincristine, etoposide, prednisone]). Long-term treatment results depends on a subtype and other prognostic factors. About 40–65% patients with ALCL ALK(–) develop reccurent disease. High-dose chemotherapy and autologous hematopoietic stem cell trasplantation improve outcome in this group of patients. Brentuximab vedotin has shown efficacy with relapsed sALCL and enable gaining objective respones in 86% of cases.
{"title":"Zastosowanie brentuksymabu vedotin w trzeciej linii leczenia ch orego na pierwotnie opornego układowego chłoniaka anaplastycznego z dużych komórek ALK(–)","authors":"Beata Kania-Zembaczyńska","doi":"10.5603/hem.2019.0021","DOIUrl":"https://doi.org/10.5603/hem.2019.0021","url":null,"abstract":"Anaplastic large cell lymphoma (ALCL) is rare hematological malignancy and subtype of mature T-cell lymphoma. Most patients with diagnosed systemic ALCL (sALCL) respond to common first-line chemotherapy based on antracycline (CHOP [doxorubicin, vincristine, prednisone], or CHOEP [doxorubicin, vincristine, etoposide, prednisone]). Long-term treatment results depends on a subtype and other prognostic factors. About 40–65% patients with ALCL ALK(–) develop reccurent disease. High-dose chemotherapy and autologous hematopoietic stem cell trasplantation improve outcome in this group of patients. Brentuximab vedotin has shown efficacy with relapsed sALCL and enable gaining objective respones in 86% of cases.","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":"88338558","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}
As an inhibitor of JAK1/JAK2 kinase, ruxolitinib is an effective drug for the treatment of patients with primary and secondary myelofibrosis. Around half such patients demonstrate recovery from the disease’s general symptoms and have their enlarged spleens reduced, together with having an extended survival time. Nevertheless, some patients suffer side effects which thus indicate that the drug is discontinued. In some cases a sudden discontinuation leads to ruxolitinib withdrawal syndrome presenting as a rapid relapse back to the disease symptoms before treatment, as well as rapid splenomegaly. Also rarely occurring, are severe haemodynamic disturbances requiring intensive care. We hereby present a case report of a patient in whom ruxolitinib was discontinued resulting in withdrawal syndrome appearance.
{"title":"Zespół odstawienia ruksolitynibu u chorej leczonej z powodu mielofibrozy","authors":"I. Seferynska, K. Warzocha","doi":"10.5603/EM.2019.0012","DOIUrl":"https://doi.org/10.5603/EM.2019.0012","url":null,"abstract":"As an inhibitor of JAK1/JAK2 kinase, ruxolitinib is an effective drug for the treatment of patients with primary and secondary myelofibrosis. Around half such patients demonstrate recovery from the disease’s general symptoms and have their enlarged spleens reduced, together with having an extended survival time. Nevertheless, some patients suffer side effects which thus indicate that the drug is discontinued. In some cases a sudden discontinuation leads to ruxolitinib withdrawal syndrome presenting as a rapid relapse back to the disease symptoms before treatment, as well as rapid splenomegaly. Also rarely occurring, are severe haemodynamic disturbances requiring intensive care. We hereby present a case report of a patient in whom ruxolitinib was discontinued resulting in withdrawal syndrome appearance.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-08-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85185022","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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