46-years old male diagnosed with primary myelofibrosis (PMF) was included into therapeutic program of treatment with ruxolitinib. Due to initial thrombocytopenia with basal platelets [PLT] range below 100 G/l, initial dosage of the treatment was reduced according to characteristics of drug. No treatment-related toxicity was noted, the dosage was gradually increased reaching the dose 20 mg twice a day at twelfth week of therapy. Although alleviation of systemic symptoms was reached, lack of diminishment of spleen size demanded by therapeutic program led to cessation of treatment. In group of patients with PMF and secondary thrombocytopenia treatment with ruxolitinib may lead to improvement of patient condition.
{"title":"Leczenie ruksolitynibem u chorego na pierwotne włóknienie szpiku oraz małopłytkowość","authors":"P. Chrząstek, Dariusz Woszczyk","doi":"10.5603/HEM.2019.0009","DOIUrl":"https://doi.org/10.5603/HEM.2019.0009","url":null,"abstract":"46-years old male diagnosed with primary myelofibrosis (PMF) was included into therapeutic \u0000program of treatment with ruxolitinib. Due to initial thrombocytopenia with basal platelets [PLT] \u0000range below 100 G/l, initial dosage of the treatment was reduced according to characteristics of \u0000drug. No treatment-related toxicity was noted, the dosage was gradually increased reaching the \u0000dose 20 mg twice a day at twelfth week of therapy. Although alleviation of systemic symptoms was \u0000reached, lack of diminishment of spleen size demanded by therapeutic program led to cessation of \u0000treatment. In group of patients with PMF and secondary thrombocytopenia treatment with ruxolitinib \u0000may lead to improvement of patient condition.","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":"82365145","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. Sobas, A. Czyż, Krzysztof Zduniak, M. Biedroń, T. Wróbel
Ruxolitynib — JAK1/JAK2 inhibitor — registrated for patients with myelofibrosis (MF) IPSS (International Prognostic Scoring System) intermediate-2 or high, with splenomegaly and general symptomatology. Moreover, ruxolitinib is used to reduce splenomegaly and general symptomatology in patients with MF who are candidates for allogeneic hematopoietic stem cells transplantation (allo- -HSCT). Anemia, thrombocytopenia and occasionally leukopenia with neutropenia are observed as a main side effects of ruxolitinib. Moreover, there are problems related to cytokine storm caused by treatment discontinuation. Here we report the patient with MF and important leucocytosis, in whom splenomegaly and general symptoms were reduced due to ruxolitinib. However, ruxolitinib in monotherapy did not reduce the leukocyte count, so the the combined therapy with ruxolitinib plus hydroxycarbamide was initiated. As the result of the treatment, reduction of number of leukocytes was observed, meanwhile the level of hemoglobin was stable and platelets count drop slightly. Thereafter, the patient proceeded to allo-HSCT. So far, there are two cases of patients treated with ruxolitinib plus hydroxycarbamid published in the literature. We think, that all cases treated in that way, should be reporteds, as we can establish the safety of this drug combination.
{"title":"Problem kontroli leukocytozy u chorego na pierwotne włóknienie szpiku leczonego ruksolitynibem","authors":"M. Sobas, A. Czyż, Krzysztof Zduniak, M. Biedroń, T. Wróbel","doi":"10.5603/HEM.2019.0006","DOIUrl":"https://doi.org/10.5603/HEM.2019.0006","url":null,"abstract":"Ruxolitynib — JAK1/JAK2 inhibitor — registrated for patients with myelofibrosis (MF) IPSS (International \u0000Prognostic Scoring System) intermediate-2 or high, with splenomegaly and general symptomatology. \u0000Moreover, ruxolitinib is used to reduce splenomegaly and general symptomatology in \u0000patients with MF who are candidates for allogeneic hematopoietic stem cells transplantation (allo- \u0000-HSCT). Anemia, thrombocytopenia and occasionally leukopenia with neutropenia are observed \u0000as a main side effects of ruxolitinib. Moreover, there are problems related to cytokine storm caused \u0000by treatment discontinuation. Here we report the patient with MF and important leucocytosis, in whom splenomegaly and general symptoms were reduced due to ruxolitinib. However, ruxolitinib \u0000in monotherapy did not reduce the leukocyte count, so the the combined therapy with ruxolitinib \u0000plus hydroxycarbamide was initiated. As the result of the treatment, reduction of number of leukocytes \u0000was observed, meanwhile the level of hemoglobin was stable and platelets count drop slightly. \u0000Thereafter, the patient proceeded to allo-HSCT. So far, there are two cases of patients treated with \u0000ruxolitinib plus hydroxycarbamid published in the literature. We think, that all cases treated in that \u0000way, should be reporteds, as we can establish the safety of this drug combination.","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":"73923964","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}
Primary myelofibrosis belongs to chronic myeloproliferative neoplasms BCR-ABL1-negative. In this article, we present a case of 74 years-old woman, treated with hydroxyurea at the beginning of the diagnosis. Subsequently, ruxolitinib therapy was started.
{"title":"Skuteczność terapii ruksolitynibem u chorej na pierwotne włóknienie szpiku","authors":"A. Obara, Marcin Pasiarski","doi":"10.5603/HEM.2019.0010","DOIUrl":"https://doi.org/10.5603/HEM.2019.0010","url":null,"abstract":"Primary myelofibrosis belongs to chronic myeloproliferative neoplasms BCR-ABL1-negative. In this article, we present a case of 74 years-old woman, treated with hydroxyurea at the beginning of the diagnosis. Subsequently, ruxolitinib therapy was started.","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":"73947997","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":"Skuteczność zastosowania ruksolitynibu u chorej z wtórnym włóknieniem szpiku w przebiegu wieloletniej czerwienicy prawdziwej powikłanej zakrzepicą w układzie wrotnym","authors":"E. Bodzenta, S. Grosicki","doi":"10.5603/hem.2019.0007","DOIUrl":"https://doi.org/10.5603/hem.2019.0007","url":null,"abstract":"","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":"81867289","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}
Philadelphia-negative myeloproliferative neoplasms are associated with increased risk of lymphoid neoplasms. In this article a female patient with essential thrombocythemia and Hodgkin lymphoma was reported. When remission of lymphoma was obtained secondary myelofibrosis was developed. Therapy with ruxolitinib resulted in resolution of constitutional symptoms and reduction of spleen volume in a short period of time.
{"title":"Współistnienie chłoniaka Hodgkina i mielofibrozy u chorej leczonej ruksolitynibem","authors":"M. Cioch","doi":"10.5603/HEM.2019.0008","DOIUrl":"https://doi.org/10.5603/HEM.2019.0008","url":null,"abstract":"Philadelphia-negative myeloproliferative neoplasms are associated with increased risk of lymphoid neoplasms. In this article a female patient with essential thrombocythemia and Hodgkin lymphoma was reported. When remission of lymphoma was obtained secondary myelofibrosis was developed. Therapy with ruxolitinib resulted in resolution of constitutional symptoms and reduction of spleen volume in a short period of time.","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":"85721582","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. Suska, Dorota Rowczenio, J. Jaszczyński, Wojciech Szot, Sarah Goldman-Mazur, A. Jurczyszyn
Amyloidosis is a heterogeneous group of protein misfolding diseases caused by extracellular deposition of abnormal beta-fibrils resistant to proteolysis. The most common type is light-chain amyloidosis (AL amyloidosis) which can be systemic or localized. Localized amyloidosis mostly affects the respiratory airways, genitourinary tract, gastrointestinal system or skin. We present diagnostic and therapeutic approach in a 25-year-old female patient diagnosed with urinary bladder amyloidosis. The main complaint was macroscopic hematuria occurring periodically for 2 years. Abdominal and pelvic computed tomography revealed single 25-mm-thick soft-tissue lesion of the left bladder wall. Histopathological examination of the lesion biopsied during transurethral resection of bladder tumor showed amyloid deposits with strong positive immunostaining for transthyretin, weaker for light chain (AL) and weak for serum amyloid A (AA). Serum protein electrophoresis and immunofixation did not reveal monoclonal protein. X-rays of flat bones presented without lytic lesions. There were no amyloid deposits both in trephine biopsy and subcutaneous fat biopsy. Primary systemic AL amyloidosis was excluded. According to the results of (99m)Tc-DPD scintigraphy and genetic analysis of transthyretin gene (TTR), the ATTRm amyloidosis was also excluded. Consultative histopathological analysis of the bladder biopsy made in the National Amyloidosis Center in London revealed amyloid deposits stained to lambda light chains, confirming the diagnosis of localized AL lambda bladder amyloidosis. The patient was qualified for surgical treatment. Partial resection of the bladder wall with no need for left ureter transplantation was performed. Primary localized bladder amyloidosis is a very rare entity. In the diagnostic approach the most important is the exclusion of primary systemic amyloidosis due to a completely different treatment method.
{"title":"Amyloidoza pęcherza moczowego — opis przypadku i przegląd piśmiennictwa","authors":"A. Suska, Dorota Rowczenio, J. Jaszczyński, Wojciech Szot, Sarah Goldman-Mazur, A. Jurczyszyn","doi":"10.5603/HEM.2018.0039","DOIUrl":"https://doi.org/10.5603/HEM.2018.0039","url":null,"abstract":"Amyloidosis is a heterogeneous group of protein misfolding diseases caused by extracellular deposition \u0000of abnormal beta-fibrils resistant to proteolysis. The most common type is light-chain amyloidosis \u0000(AL amyloidosis) which can be systemic or localized. Localized amyloidosis mostly affects the \u0000respiratory airways, genitourinary tract, gastrointestinal system or skin. We present diagnostic and \u0000therapeutic approach in a 25-year-old female patient diagnosed with urinary bladder amyloidosis. \u0000The main complaint was macroscopic hematuria occurring periodically for 2 years. Abdominal \u0000and pelvic computed tomography revealed single 25-mm-thick soft-tissue lesion of the left bladder \u0000wall. Histopathological examination of the lesion biopsied during transurethral resection of bladder \u0000tumor showed amyloid deposits with strong positive immunostaining for transthyretin, weaker for \u0000light chain (AL) and weak for serum amyloid A (AA). Serum protein electrophoresis and immunofixation \u0000did not reveal monoclonal protein. X-rays of flat bones presented without lytic lesions. There \u0000were no amyloid deposits both in trephine biopsy and subcutaneous fat biopsy. Primary systemic \u0000AL amyloidosis was excluded. According to the results of (99m)Tc-DPD scintigraphy and genetic \u0000analysis of transthyretin gene (TTR), the ATTRm amyloidosis was also excluded. Consultative histopathological \u0000analysis of the bladder biopsy made in the National Amyloidosis Center in London \u0000revealed amyloid deposits stained to lambda light chains, confirming the diagnosis of localized AL \u0000lambda bladder amyloidosis. The patient was qualified for surgical treatment. Partial resection of \u0000the bladder wall with no need for left ureter transplantation was performed. Primary localized bladder \u0000amyloidosis is a very rare entity. In the diagnostic approach the most important is the exclusion \u0000of primary systemic amyloidosis due to a completely different treatment method.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74177235","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}
K. Warzocha, W. Homenda, A. Pluta, Tomasz Sacha, M. Cioch, M. Dudziński, D. Krochmalczyk, J. Góra-Tybor, I. Seferynska, M. Joks, M. Sobas
{"title":"Ruksolitynib w terapii chorych na mielofibrozę — pytania i odpowiedzi","authors":"K. Warzocha, W. Homenda, A. Pluta, Tomasz Sacha, M. Cioch, M. Dudziński, D. Krochmalczyk, J. Góra-Tybor, I. Seferynska, M. Joks, M. Sobas","doi":"10.5603/HEM.2018.0035","DOIUrl":"https://doi.org/10.5603/HEM.2018.0035","url":null,"abstract":"","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86091363","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}
Plasmapheresis is a procedure which performed for appropriate indications contributes to the improvement of patients’ clinical condition and treatment prognosis. Plasmapheresis is also one of the therapy methods used in acute phases of autoimmune disorders. Hereby, the theoretical and practical possibilities offered by therapeutic plasma exchange in supportive treatment of various medical conditions are described. The article is based on the reports of procedures performed at the Department of Transfusion Medicine of the Institute of Hematology and Transfusion Medicine in the period 2016–2017. It presents the mechanism of plasmapheresis, the methods used in everyday practice as well as the effect of the procedure on blood components and plasma. It also emphasizes the significant role of the nurse in the plasmapheresis procedure particularly in relation to possible complications and adverse reactions.
{"title":"Terapeutyczna plazmafereza w praktyce klinicznej","authors":"B. Wiśniewska","doi":"10.5603/HEM.2018.0037","DOIUrl":"https://doi.org/10.5603/HEM.2018.0037","url":null,"abstract":"Plasmapheresis is a procedure which performed for appropriate indications contributes to the improvement of patients’ clinical condition and treatment prognosis. Plasmapheresis is also one of the therapy methods used in acute phases of autoimmune disorders. Hereby, the theoretical and practical possibilities offered by therapeutic plasma exchange in supportive treatment of various medical conditions are described. The article is based on the reports of procedures performed at the Department of Transfusion Medicine of the Institute of Hematology and Transfusion Medicine in the period 2016–2017. It presents the mechanism of plasmapheresis, the methods used in everyday practice as well as the effect of the procedure on blood components and plasma. It also emphasizes the significant role of the nurse in the plasmapheresis procedure particularly in relation to possible complications and adverse reactions.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87618481","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}
We present a 69-year-old patient, in whom in March 2016 a monoclonal gammopathy of undetermined significance (MGUS) was diagnosed. The disease was found during the diagnostics of thrombocytopenia (from 2009 the number of platelets varied between 90–140 G/l). In serum immunofixation assay the presence of monoclonal IgG kappa protein was confirmed. In July 2017, when the patient presented with nephrotic syndrome and acute renal failure, 14.5% of plasma cells were found in the bone marrow. Moreover, a biopsy of abdominal fat revealed the presence of amyloid composed of kappa light chains. The diagnosis of IgG kappa plasma cell myeloma (PCM) complicated with amyloidosis of the kidneys, heart, liver and spleen was established. The PCM progression complicated with amyloidosis was very aggressive. Despite the treatment with bortezomib and dexamethasone the patient died early. Sudden deterioration of health (e.g. in the form of acute renal failure) in a patient with MGUS may indicate not only the progression of MGUS to PCM, but also the development of amyloidosis, which is associated with turbulent disease and poor prognosis.
{"title":"Od monoklonalnej gammapatii o nieokreślonym znaczeniu (MGUS) do szpiczaka plazmocytowego i amyloidozy — opis przypadku","authors":"Michał Bator, S. Potoczek, L. Usnarska-Zubkiewicz","doi":"10.5603/HEM.2018.0040","DOIUrl":"https://doi.org/10.5603/HEM.2018.0040","url":null,"abstract":"We present a 69-year-old patient, in whom in March 2016 a monoclonal gammopathy of undetermined significance (MGUS) was diagnosed. The disease was found during the diagnostics of thrombocytopenia (from 2009 the number of platelets varied between 90–140 G/l). In serum immunofixation assay the presence of monoclonal IgG kappa protein was confirmed. In July 2017, when the patient presented with nephrotic syndrome and acute renal failure, 14.5% of plasma cells were found in the bone marrow. Moreover, a biopsy of abdominal fat revealed the presence of amyloid composed of kappa light chains. The diagnosis of IgG kappa plasma cell myeloma (PCM) complicated with amyloidosis of the kidneys, heart, liver and spleen was established. The PCM progression complicated with amyloidosis was very aggressive. Despite the treatment with bortezomib and dexamethasone the patient died early. Sudden deterioration of health (e.g. in the form of acute renal failure) in a patient with MGUS may indicate not only the progression of MGUS to PCM, but also the development of amyloidosis, which is associated with turbulent disease and poor prognosis.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2019-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73877859","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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