The biannual Congress of the Polish Society of Hematology and Transfusion Medicine is the most important event for all those involved in hematology and transfusiology. Between 14 and 16 September 2023, almost 1,300 attendees met in Katowice to discuss progress in these disciplines. Nowadays, the results of therapy of hematological malignancies in Poland are almost the same as the European average value. This is the result of hard work by the national working groups as well as international collaboration. Since 2021, Polish hematology has achieved great progress in therapy with the use of chimeric antigen receptor T-cell therapy. Also, a number of new compounds for hematological disorders, and supportive therapy, have been introduced in Poland, either within clinical trials or reimbursed by the National Health Fund. Even so, many patients still suffer unmet medical needs. Several important compounds, already licensed, are awaiting reimbursement. New cellular technologies are in the pipeline. The reimbursement of more therapies is desired, and further progress in diagnostic and therapeutic methods is required.
{"title":"Towards the future of Polish hematology: perspectives after 31st Meeting of Polish Society of Hematologists and Transfusiologists","authors":"Jan Styczyński","doi":"10.5603/ahp.97715","DOIUrl":"https://doi.org/10.5603/ahp.97715","url":null,"abstract":"The biannual Congress of the Polish Society of Hematology and Transfusion Medicine is the most important event for all those involved in hematology and transfusiology. Between 14 and 16 September 2023, almost 1,300 attendees met in Katowice to discuss progress in these disciplines. Nowadays, the results of therapy of hematological malignancies in Poland are almost the same as the European average value. This is the result of hard work by the national working groups as well as international collaboration. Since 2021, Polish hematology has achieved great progress in therapy with the use of chimeric antigen receptor T-cell therapy. Also, a number of new compounds for hematological disorders, and supportive therapy, have been introduced in Poland, either within clinical trials or reimbursed by the National Health Fund. Even so, many patients still suffer unmet medical needs. Several important compounds, already licensed, are awaiting reimbursement. New cellular technologies are in the pipeline. The reimbursement of more therapies is desired, and further progress in diagnostic and therapeutic methods is required.","PeriodicalId":35805,"journal":{"name":"Acta Haematologica Polonica","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136294119","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}
Melissa Prodisteanu, Artur Tumiński, Monika Richert-Przygońska, Robert Dębski, Krzysztof Czyżewski
{"title":"Prophylaxis with tenofovir in hepatitis-associated severe aplastic anemia treated with hematopoietic cell transplantation","authors":"Melissa Prodisteanu, Artur Tumiński, Monika Richert-Przygońska, Robert Dębski, Krzysztof Czyżewski","doi":"10.5603/ahp.96892","DOIUrl":"https://doi.org/10.5603/ahp.96892","url":null,"abstract":"","PeriodicalId":35805,"journal":{"name":"Acta Haematologica Polonica","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136356746","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}
Anna Raciborska, Zofia Małas, Andrzej Tysarowski, Katarzyna Seliga, Katarzyna Machnik, Jadwiga Wecławek-Tompol, Katarzyna Derwich, Iwona Ruranska, Katarzyna Muszyńska-Rosłan, Elżbieta Michalak, Wanda Badowska, Grażyna Karolczyk, Radosław Chaber, Katarzyna Drabko, Carlos Rodriguez-Galindo
{"title":"Impact of molecular status on clinical management in children with histiocytosis treated according to POL HISTIO project","authors":"Anna Raciborska, Zofia Małas, Andrzej Tysarowski, Katarzyna Seliga, Katarzyna Machnik, Jadwiga Wecławek-Tompol, Katarzyna Derwich, Iwona Ruranska, Katarzyna Muszyńska-Rosłan, Elżbieta Michalak, Wanda Badowska, Grażyna Karolczyk, Radosław Chaber, Katarzyna Drabko, Carlos Rodriguez-Galindo","doi":"10.5603/ahp.95452","DOIUrl":"https://doi.org/10.5603/ahp.95452","url":null,"abstract":"","PeriodicalId":35805,"journal":{"name":"Acta Haematologica Polonica","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135480827","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}
Piotr Homa, Wojciech Homa, Urszula Janiuk, Magdalena Cienkusz, Teresa Bielecka, Katarzyna Krenke, Katarzyna Drabko
In this article, we report a case of a 6-year-old girl, initially diagnosed with myelodysplastic syndrome (MDS), who presented a compound and uncharacteristic set of symptoms that included low level of lymphocytes, hypogammaglobulinemia, developmental delay and facial anomalies. An unconventional course of the disease gave premises to extended genetic diagnostic using next-generation sequencing, that provided the result of heterozygous ZBTB24 c.[1222T > G] cysteine-to-glycine missense mutation, which is characteristic of immunodeficiency, centromeric instability and facial dysmorphism syndrome type 2 (ICF2). During the treatment of hematologic disorders, the patient underwent allogenic stem cell transplantation (allo-HSCT). After performing allo-HSCT, another rare syndrome has been developed in form of pulmonary alveolar proteinosis (PAP). In this paper, we describe the whole diagnostic process, usage of drugs that include immunoglobulins, rituximab, steroids, azathioprine and performed therapeutic procedures, such as allo-HSCT and lung lavages. The unique value of this case lays in the coexistence of several rare diseases and response of the patient to multi-thread treatment.
{"title":"Myelodysplastic syndrome and pulmonary alveolar proteinosis in a 6-year-old girl with mutation of the ZBTB24 gene","authors":"Piotr Homa, Wojciech Homa, Urszula Janiuk, Magdalena Cienkusz, Teresa Bielecka, Katarzyna Krenke, Katarzyna Drabko","doi":"10.5603/ahp.95371","DOIUrl":"https://doi.org/10.5603/ahp.95371","url":null,"abstract":"In this article, we report a case of a 6-year-old girl, initially diagnosed with myelodysplastic syndrome (MDS), who presented a compound and uncharacteristic set of symptoms that included low level of lymphocytes, hypogammaglobulinemia, developmental delay and facial anomalies. An unconventional course of the disease gave premises to extended genetic diagnostic using next-generation sequencing, that provided the result of heterozygous ZBTB24 c.[1222T > G] cysteine-to-glycine missense mutation, which is characteristic of immunodeficiency, centromeric instability and facial dysmorphism syndrome type 2 (ICF2). During the treatment of hematologic disorders, the patient underwent allogenic stem cell transplantation (allo-HSCT). After performing allo-HSCT, another rare syndrome has been developed in form of pulmonary alveolar proteinosis (PAP). In this paper, we describe the whole diagnostic process, usage of drugs that include immunoglobulins, rituximab, steroids, azathioprine and performed therapeutic procedures, such as allo-HSCT and lung lavages. The unique value of this case lays in the coexistence of several rare diseases and response of the patient to multi-thread treatment.","PeriodicalId":35805,"journal":{"name":"Acta Haematologica Polonica","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135435855","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}
Monika Richert-Przygońska, Krzysztof Czyżewski, Robert Dębski, Małgorzata Kubicka, Beata Kurylo-Rafińka, Agnieszka Majk, Ewa Dembna, Łukasz Ledziński, Ewa Marquardt, Honorata Mlicka, Katarzyna Gągola, Jan Styczyński
{"title":"Very late relapse of ALL after 14 years: treatment with CAR-T cells","authors":"Monika Richert-Przygońska, Krzysztof Czyżewski, Robert Dębski, Małgorzata Kubicka, Beata Kurylo-Rafińka, Agnieszka Majk, Ewa Dembna, Łukasz Ledziński, Ewa Marquardt, Honorata Mlicka, Katarzyna Gągola, Jan Styczyński","doi":"10.5603/ahp.96850","DOIUrl":"https://doi.org/10.5603/ahp.96850","url":null,"abstract":"","PeriodicalId":35805,"journal":{"name":"Acta Haematologica Polonica","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135482517","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}
Monika Richert-Przygońska, Magdalena Milewska, Robert Dębski, Joanna Cisek, Krzysztof Czyżewski
Chronic graft versus host disease (cGHVD) is one of the most serious complications after allogeneic hematopoietic cell transplantation (allo-HCT). It varies in between patients, often leading to systemic and functional limitations. It can be challenging considering the heterogeneity of patients. The objective of this paper is to present clinical aspects of sclerodermatous manifestation of cGVHD as a complication of allo-HCT in pediatric patients. We diagnosed three patients with different sclerodermatous skin involvement and cGVHD features. The treatment applied varied among patients and was based on current available standards of care. We analyzed effectiveness of systemic steroid therapy, extracorporeal photopheresis and ruxolitinib. In conclusion, all patients achieved improvement in skin leasions and quality of life, based on the individualized treatment approach.
{"title":"Sclerodermatous manifestation of chronic graft versus host disease: therapy challenges","authors":"Monika Richert-Przygońska, Magdalena Milewska, Robert Dębski, Joanna Cisek, Krzysztof Czyżewski","doi":"10.5603/ahp.96817","DOIUrl":"https://doi.org/10.5603/ahp.96817","url":null,"abstract":"Chronic graft versus host disease (cGHVD) is one of the most serious complications after allogeneic hematopoietic cell transplantation (allo-HCT). It varies in between patients, often leading to systemic and functional limitations. It can be challenging considering the heterogeneity of patients. The objective of this paper is to present clinical aspects of sclerodermatous manifestation of cGVHD as a complication of allo-HCT in pediatric patients. We diagnosed three patients with different sclerodermatous skin involvement and cGVHD features. The treatment applied varied among patients and was based on current available standards of care. We analyzed effectiveness of systemic steroid therapy, extracorporeal photopheresis and ruxolitinib. In conclusion, all patients achieved improvement in skin leasions and quality of life, based on the individualized treatment approach.","PeriodicalId":35805,"journal":{"name":"Acta Haematologica Polonica","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134975025","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}
Dyskeratosis congenita (DC) is a rare multisystem clinical entity caused by genetic mutations associated with telomere biology disorder. The symptoms include bone marrow dysfunction as well as skin and mucosal abnormalities. In severe cases, DC is characterized by high mortality rates among children. In milder subtypes, it is less detectable in adults, due to the occurrence of cryptic forms of the disease. To date, more than 15 mutated genes have been shown as causative for DC. The aim of this study was to provide a brief description of the currently known clinical and genetic characteristics of DC, and to elucidate the molecular pathogenesis.
{"title":"Dyskeratosis congenita as a multifaceted bone marrow disorder","authors":"Maciej Mazurek, Joanna Madzio, Wojciech Młynarski","doi":"10.5603/ahp.95640","DOIUrl":"https://doi.org/10.5603/ahp.95640","url":null,"abstract":"Dyskeratosis congenita (DC) is a rare multisystem clinical entity caused by genetic mutations associated with telomere biology disorder. The symptoms include bone marrow dysfunction as well as skin and mucosal abnormalities. In severe cases, DC is characterized by high mortality rates among children. In milder subtypes, it is less detectable in adults, due to the occurrence of cryptic forms of the disease. To date, more than 15 mutated genes have been shown as causative for DC. The aim of this study was to provide a brief description of the currently known clinical and genetic characteristics of DC, and to elucidate the molecular pathogenesis.","PeriodicalId":35805,"journal":{"name":"Acta Haematologica Polonica","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135695705","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":"Hematology and transfusion medicine journal of pediatric and adult diseases: we all are readers, authors and reviewers","authors":"Jan Styczyński","doi":"10.5603/ahp.97593","DOIUrl":"https://doi.org/10.5603/ahp.97593","url":null,"abstract":"","PeriodicalId":35805,"journal":{"name":"Acta Haematologica Polonica","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135696249","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}
Alicja Maziarczyk, Maciej Lambach, Paulina Kura, Monika Lejman, Joanna Zawitkowska
One of the most common causes of acute pancreatitis in children is medications. These include L-asparaginase, glucocorticoids and 6-mercaptopurine, which are widely used in the therapy of acute lymphoblastic leukemia (ALL). When L-asparaginase and glucocorticoids are administered together, blood triglyceride levels increase, which consequently further enhances the risk of pancreatitis. Therefore, acute pancreatitis is a common side effect of ALL treatment, present in 2.3–11% of pediatric patients. The aim of this paper was to review studies on acute pancreatitis in children with ALL and to assess potential risk factors, treatment outcomes and recurrence of this condition. Based on the studies conducted, we found potential risk factors, other than the drugs mentioned above, to be the patient’s age at diagnosis, obesity, the type of L-asparaginase administered, and the cumulative or peak dose of L-asparaginase or other drug used. Fortunately, the course of pancreatitis is usually mild to moderate, and the treatment is mainly symptomatic. Moreover, a successful treatment option may be octreotide. As children who have received less than 25 weeks of L-asparaginase therapy have presented with inferior outcomes, it seems reasonable to reintroduce this drug into ALL treatment after an episode of pancreatitis. The incidence of recurrent pancreatitis after re-treatment with L-asparaginase varies depending on the study. Unfortunately, the outcomes for children who develop acute pancreatitis during ALL treatment are usually worse compared to children without an acute pancreatitis history, but the results remain inconclusive. Further research is needed to assess the management of acute pancreatitis, and to minimize the worsening of ALL outcomes among these patients.
{"title":"Acute pancreatitis in children with acute lymphoblastic leukemia","authors":"Alicja Maziarczyk, Maciej Lambach, Paulina Kura, Monika Lejman, Joanna Zawitkowska","doi":"10.5603/ahp.95319","DOIUrl":"https://doi.org/10.5603/ahp.95319","url":null,"abstract":"One of the most common causes of acute pancreatitis in children is medications. These include L-asparaginase, glucocorticoids and 6-mercaptopurine, which are widely used in the therapy of acute lymphoblastic leukemia (ALL). When L-asparaginase and glucocorticoids are administered together, blood triglyceride levels increase, which consequently further enhances the risk of pancreatitis. Therefore, acute pancreatitis is a common side effect of ALL treatment, present in 2.3–11% of pediatric patients. The aim of this paper was to review studies on acute pancreatitis in children with ALL and to assess potential risk factors, treatment outcomes and recurrence of this condition. Based on the studies conducted, we found potential risk factors, other than the drugs mentioned above, to be the patient’s age at diagnosis, obesity, the type of L-asparaginase administered, and the cumulative or peak dose of L-asparaginase or other drug used. Fortunately, the course of pancreatitis is usually mild to moderate, and the treatment is mainly symptomatic. Moreover, a successful treatment option may be octreotide. As children who have received less than 25 weeks of L-asparaginase therapy have presented with inferior outcomes, it seems reasonable to reintroduce this drug into ALL treatment after an episode of pancreatitis. The incidence of recurrent pancreatitis after re-treatment with L-asparaginase varies depending on the study. Unfortunately, the outcomes for children who develop acute pancreatitis during ALL treatment are usually worse compared to children without an acute pancreatitis history, but the results remain inconclusive. Further research is needed to assess the management of acute pancreatitis, and to minimize the worsening of ALL outcomes among these patients.","PeriodicalId":35805,"journal":{"name":"Acta Haematologica Polonica","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135696517","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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