Waldenström macroglobulinemia (WM), according to the 2017 World Health Organization classifi - cation, is defined as the co-occurrence of lymphoplasmacytic lymphoma involving the bone marrow with monoclonal gammopathy of the IgM class regardless of the concentration of monoclonal pro - tein. It is a rare lymphoproliferative disease with distinctive clinical features. Diagnostic character - istics in WM have changed significantly with the discovery of two molecular markers: MYD88 and CXCR4. The mutational status of these markers both affects clinical presentation and has shown therapeutic implications. The choice of treatment in WM is closely dependent on the patient’s age, risk of treatment-related neuropathy, and risk of immunosuppression or secondary malignancies. The therapeutic landscape has broadened in recent years, and the approvals of ibrutinib and zanu - brutinib represent a significant step forward toward better management of the disease
{"title":"Waldenström macroglobulinemia: diagnosis and treatment","authors":"K. Giannopoulos","doi":"10.5603/hcp.a2022.0014","DOIUrl":"https://doi.org/10.5603/hcp.a2022.0014","url":null,"abstract":"Waldenström macroglobulinemia (WM), according to the 2017 World Health Organization classifi - cation, is defined as the co-occurrence of lymphoplasmacytic lymphoma involving the bone marrow with monoclonal gammopathy of the IgM class regardless of the concentration of monoclonal pro - tein. It is a rare lymphoproliferative disease with distinctive clinical features. Diagnostic character - istics in WM have changed significantly with the discovery of two molecular markers: MYD88 and CXCR4. The mutational status of these markers both affects clinical presentation and has shown therapeutic implications. The choice of treatment in WM is closely dependent on the patient’s age, risk of treatment-related neuropathy, and risk of immunosuppression or secondary malignancies. The therapeutic landscape has broadened in recent years, and the approvals of ibrutinib and zanu - brutinib represent a significant step forward toward better management of the disease","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78817583","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}
Krzysztof Tomasiewicz, Alicja Dębska-Ślizień, Magdalena Durlik, Krzysztof Giannopoulos, Iwona Hus, Piotr Rutkowski
{"title":"Recommendations for prevention of SARS-CoV-2 infection in immunocompromised patients","authors":"Krzysztof Tomasiewicz, Alicja Dębska-Ślizień, Magdalena Durlik, Krzysztof Giannopoulos, Iwona Hus, Piotr Rutkowski","doi":"10.5603/hcp.a2022.0012","DOIUrl":"https://doi.org/10.5603/hcp.a2022.0012","url":null,"abstract":"","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135306822","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}
Daria Majowicz, K. Kostrzewa, Tomasz Gromek, B. Sokołowska, M. Hus
{"title":"Haemophagocytic lymphohistiocytosis: case series. Serum ferritin level as an indicator of treatment effectiveness","authors":"Daria Majowicz, K. Kostrzewa, Tomasz Gromek, B. Sokołowska, M. Hus","doi":"10.5603/hcp.a2022.0015","DOIUrl":"https://doi.org/10.5603/hcp.a2022.0015","url":null,"abstract":"","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-01-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73901723","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}
Immune thrombocytopenia (ITP) is an acquired autoimmune disease characterized by an isolated platelet count of less than 100,000/μL caused by destruction and reduced production of platelets. Systemic steroid therapy is a mainstay of first-line treatment; however, additional treatment lines are usually necessary due to the very high recurrence rate after therapy completion and steroid resistance. Standard treatment options for steroid resistance or steroid dependence include splenectomy, rituximab or thrombopoietin receptor agonists. The article presents current data on the diagnosics and therapy of ITP, considering the limited access to some forms of treatment in Poland
{"title":"Diagnosis and treatment of immune thrombocytopenia in Poland","authors":"P. Malecki, S. Grosicki","doi":"10.5603/hcp.a2022.0013","DOIUrl":"https://doi.org/10.5603/hcp.a2022.0013","url":null,"abstract":"Immune thrombocytopenia (ITP) is an acquired autoimmune disease characterized by an isolated platelet count of less than 100,000/μL caused by destruction and reduced production of platelets. Systemic steroid therapy is a mainstay of first-line treatment; however, additional treatment lines are usually necessary due to the very high recurrence rate after therapy completion and steroid resistance. Standard treatment options for steroid resistance or steroid dependence include splenectomy, rituximab or thrombopoietin receptor agonists. The article presents current data on the diagnosics and therapy of ITP, considering the limited access to some forms of treatment in Poland","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75258273","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. Giannopoulos, K. Jamroziak, T. Wróbel, D. Dytfeld
Plasma cell myeloma (PCM) is a hematologic malignancy that derives from mature B cells. The prognosis of patients with PCM is highly dependent on the presence of cytogenetic aberrations. Determination of cytogenetic risk enables informing patients about their prognosis and allows for individual choice of therapy. In Poland, cytogenetic risk assessment is a fully reimbursed procedure, and it is recommended to perform such an examination in every diagnosed patient. Therapy of patients with high cytogenetic risk should be planned with consideration of tandem autotrans-plantation of hematopoietic cells in eligible patients. In patients with refractory or relapsed PCM, treatment with ixazomib in combination with lenalidomide and dexamethasone appears to remove cytogenetic risk.
{"title":"Optimization of treatment of patients with plasma cell myeloma with high cytogenetic risk in Poland","authors":"K. Giannopoulos, K. Jamroziak, T. Wróbel, D. Dytfeld","doi":"10.5603/hcp.a2022.0007","DOIUrl":"https://doi.org/10.5603/hcp.a2022.0007","url":null,"abstract":"Plasma cell myeloma (PCM) is a hematologic malignancy that derives from mature B cells. The prognosis of patients with PCM is highly dependent on the presence of cytogenetic aberrations. Determination of cytogenetic risk enables informing patients about their prognosis and allows for individual choice of therapy. In Poland, cytogenetic risk assessment is a fully reimbursed procedure, and it is recommended to perform such an examination in every diagnosed patient. Therapy of patients with high cytogenetic risk should be planned with consideration of tandem autotrans-plantation of hematopoietic cells in eligible patients. In patients with refractory or relapsed PCM, treatment with ixazomib in combination with lenalidomide and dexamethasone appears to remove cytogenetic risk.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82058893","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 leukaemia (AML) is a complex disease with a dynamic course associated with a series of acquired and cumulative genetic changes. In recent years, significant advances have been made in the understanding of its pathogenesis. Moreover, diagnostic and therapeutic options have expanded. The current classifications consider cytogenetic and molecular disorders, including the presence of, among others, mutations within FMS-like tyrosine kinase 3 (FLT3) transmembrane tyrosine kinase, regulating the proliferation and differentiation of hematopoietic cells at an early development stage. FLT3 mutation is detected in approximately 30% of newly diagnosed AML cases and concerns mutations: internal tandem duplication (ITD) or tyrosine kinase domain (TKD) gene. The high ratio of FLT3-ITD mutation is associated with an unfavourable prognosis. It is recommended to includ patients in clinical trials due to insufficient standard therapy effects. The new AML treatment strategies include first-and second-generation tyrosine kinase inhibitors. Midostaurin, a non-specific kinase inhibitor, was approved in 2017 for treatment of patients with newly diagnosed FLT3-positive AML in combination with standard chemotherapy. The paper presents the experience of the Department of Haematology and Bone Marrow Transplantation in Poznan in the use of FLT3 tyrosine kinase inhibitors based on a case report of two patients with newly diagnosed AML.
{"title":"Midostaurin added to standard therapy in FLT3-positive acute myeloid leukaemia treatment","authors":"A. Szczepaniak, Zuzanna Rzetelska","doi":"10.5603/hcp.a2022.0011","DOIUrl":"https://doi.org/10.5603/hcp.a2022.0011","url":null,"abstract":"Acute myeloid leukaemia (AML) is a complex disease with a dynamic course associated with a series of acquired and cumulative genetic changes. In recent years, significant advances have been made in the understanding of its pathogenesis. Moreover, diagnostic and therapeutic options have expanded. The current classifications consider cytogenetic and molecular disorders, including the presence of, among others, mutations within FMS-like tyrosine kinase 3 (FLT3) transmembrane tyrosine kinase, regulating the proliferation and differentiation of hematopoietic cells at an early development stage. FLT3 mutation is detected in approximately 30% of newly diagnosed AML cases and concerns mutations: internal tandem duplication (ITD) or tyrosine kinase domain (TKD) gene. The high ratio of FLT3-ITD mutation is associated with an unfavourable prognosis. It is recommended to includ patients in clinical trials due to insufficient standard therapy effects. The new AML treatment strategies include first-and second-generation tyrosine kinase inhibitors. Midostaurin, a non-specific kinase inhibitor, was approved in 2017 for treatment of patients with newly diagnosed FLT3-positive AML in combination with standard chemotherapy. The paper presents the experience of the Department of Haematology and Bone Marrow Transplantation in Poznan in the use of FLT3 tyrosine kinase inhibitors based on a case report of two patients with newly diagnosed AML.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86480792","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}
Ewa Michalak, Agata Dudzik, Joanna Śręba, B. Kęsek, D. Darczuk
Many systemic diseases can manifest in the oral mucosa. Leukaemia is the most common neoplastic disease of white blood cells. Common symptoms of leukaemia in the oral cavity include mucosa pallor, bleeding gums, gingival enlargement, ecchymosis, oral infections and ulcerations. A dentist should know how to recognize the first signs of leukaemia and may be responsible for a prompt referral to an adequate professional to improve patient outcomes. A dentist must participate in a patient’s process of treatment when the plan includes complex therapy of the oral cavity. In many cases, this procedure enables the implementation of appropriate therapy and the possibility of recovery and can even save the patient’s life. The study aimed to present the cooperation between the dentist and haematologist in the example of a 52-year-old female with oral symptoms of leukaemia.
{"title":"Oral manifestations of leukaemia: cooperation between dentist and haematologist","authors":"Ewa Michalak, Agata Dudzik, Joanna Śręba, B. Kęsek, D. Darczuk","doi":"10.5603/hcp.a2022.0009","DOIUrl":"https://doi.org/10.5603/hcp.a2022.0009","url":null,"abstract":"Many systemic diseases can manifest in the oral mucosa. Leukaemia is the most common neoplastic disease of white blood cells. Common symptoms of leukaemia in the oral cavity include mucosa pallor, bleeding gums, gingival enlargement, ecchymosis, oral infections and ulcerations. A dentist should know how to recognize the first signs of leukaemia and may be responsible for a prompt referral to an adequate professional to improve patient outcomes. A dentist must participate in a patient’s process of treatment when the plan includes complex therapy of the oral cavity. In many cases, this procedure enables the implementation of appropriate therapy and the possibility of recovery and can even save the patient’s life. The study aimed to present the cooperation between the dentist and haematologist in the example of a 52-year-old female with oral symptoms of leukaemia.","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74971669","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}
Eosinophilia is defined as an absolute peripheral blood eosinophil count > 0.5 GL. Most often, its intensity is mild. Eosinophilia usually accompanies other diseases such as allergic, rheumatological, infectious, or oncological. The signs depend on the underlying diseases. After starting treatment of the underlying disease, the eosinophil count returns to the reference range. The diagnosis of eo-sinophilia starts by excluding the secondary causes. Clonal eosinophilia is a rare disease in which eosinophils are part of a tumor clone. The clonality is confirmed by molecular biology methods. The most common eosinophilic infiltration includes skin, lungs, and heart. The symptoms are not connected with a count of eosinophils in blood or bone marrow. The manifestation can be mild but also severe, life-threatening like venous thromboembolism. This article presents a case of 36-year-old man with eosinophilia caused by Toxocara canis infection manifested by systemic symptoms, erythroderma
{"title":"Eosinophilia caused by Toxocara canis infection","authors":"Monika Kowalik, A. Gołos, J. Góra-tybor","doi":"10.5603/hcp.a2022.0010","DOIUrl":"https://doi.org/10.5603/hcp.a2022.0010","url":null,"abstract":"Eosinophilia is defined as an absolute peripheral blood eosinophil count > 0.5 GL. Most often, its intensity is mild. Eosinophilia usually accompanies other diseases such as allergic, rheumatological, infectious, or oncological. The signs depend on the underlying diseases. After starting treatment of the underlying disease, the eosinophil count returns to the reference range. The diagnosis of eo-sinophilia starts by excluding the secondary causes. Clonal eosinophilia is a rare disease in which eosinophils are part of a tumor clone. The clonality is confirmed by molecular biology methods. The most common eosinophilic infiltration includes skin, lungs, and heart. The symptoms are not connected with a count of eosinophils in blood or bone marrow. The manifestation can be mild but also severe, life-threatening like venous thromboembolism. This article presents a case of 36-year-old man with eosinophilia caused by Toxocara canis infection manifested by systemic symptoms, erythroderma","PeriodicalId":38988,"journal":{"name":"Hematologia","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2022-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78997428","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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