Haematologia最新文献_第2页

Zastosowanie ponatynibu u pacjenta po wcześniejszym niepowodzeniu leczenia imatynibem i toksyczności dazatynibu

Haematologia Pub Date : 2020-10-30 DOI: 10.5603/HEM.2020.0012

Michał Janowski, Edyta Paczkowska

引用次数: 1

Diagnostyka i leczenie mastocytozy układowej — stanowisko ekspertów

Haematologia Pub Date : 2020-10-30 DOI: 10.5603/HEM.2020.0008

Marek Hus, Aneta Szudy-Szczyrek, G. Helbig, Tomasz Sacha, A. Mital, Bożena Katarzyna-Budziszewska, Monika Prochorec-Sobieszek, Krystyna Gałązka

{"title":"Diagnostyka i leczenie mastocytozy układowej — stanowisko ekspertów","authors":"Marek Hus, Aneta Szudy-Szczyrek, G. Helbig, Tomasz Sacha, A. Mital, Bożena Katarzyna-Budziszewska, Monika Prochorec-Sobieszek, Krystyna Gałązka","doi":"10.5603/HEM.2020.0008","DOIUrl":"https://doi.org/10.5603/HEM.2020.0008","url":null,"abstract":"Systemic mastocytosis (SM) is a rare hematological malignancy characterized by an abnormal expansion and accumulation of pathological mast cells in bone marrow and other organs including skin, liver, spleen and lymph nodes. The clinical manifestation can be extremely heterogeneous, from limited skin changes to multi-organ involvement or mast cell tumors. The median survival of patients diagnosed with indolent form is comparable to healthy population, while prognosis for patients with advanced disease is poor, with an estimated survival ranging from several months to several years. In most patients (> 90%), a somatic mutation in codon 816 of the c-KIT gene encoding tyrosine kinase receptor is detected. Additional molecular abnormalities and even coexistence of other hematological cancers, e.g . acute leukemia, are also observed. Regardless of the form of disease or serum tryptase concentration, patients are exposed to symptoms resulting from the release of mast cell mediators — most often itching, paroxysmal redness and blisters, and general mediator-induced symptoms — such as nausea, vomiting, diarrhea, abdominal pain, hypotensive episodes, fatigue, headache, fever, shortness of breath, osteopenia, osteoporosis and severe anaphylactic reactions. This paper presents current outlook on the diagnostic and treatment process of SM, taking into account the interdisciplinary aspects of the disease.","PeriodicalId":12837,"journal":{"name":"Haematologia","volume":"88 1","pages":"61-72"},"PeriodicalIF":0.0,"publicationDate":"2020-10-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90995018","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}

引用次数: 0

Pierwsza w Polsce allogeniczna transplantacja komórek macierzystych u chorego z oporną postacią białaczki włochatokomórkowej

Haematologia Pub Date : 2020-06-30 DOI: 10.5603/hem.2020.0001

M. Toborek, A. Wieczorkiewicz-Kabut, G. Semeńczuk, G. Helbig

{"title":"Pierwsza w Polsce allogeniczna transplantacja komórek macierzystych u chorego z oporną postacią białaczki włochatokomórkowej","authors":"M. Toborek, A. Wieczorkiewicz-Kabut, G. Semeńczuk, G. Helbig","doi":"10.5603/hem.2020.0001","DOIUrl":"https://doi.org/10.5603/hem.2020.0001","url":null,"abstract":"Introduction. Hairy cell leukemia (HCL) is an uncommon B-cell lymphoproliferative disorder characterized by distinctive cells present in bone marrow, peripheral blood and spleen. Treatment of relapsed and/or refractory (R/R) HCL remains a challenge. Case report. A 28-year-old male was treated with cladribine +/- rituximab for his newly diagnosed BRAF-V600 mutated HCL. A year later leukemia relapsed and he received interferon alfa with poor response. Subsequently, vemurafenib was continued for 5 months but this treatment also failed. Then, he received bendamustine with rituximab but significant response was not achieved. Finally, he was found eligible for allogeneic stem cell transplantation (Allo-SCT) from a 9/10-matched unrelated donor. He was conditioned with total body irradiation (TBI) and cyclophosphamide. Cyclosporine and methotrexate were used as graft-versus-host prophylaxis (GVHD). He engrafted and achieved full donor chimerism. There were 0.4% of hairy cells in bone marrow sample by flow cytometry at discharge. 3 months after transplantation he developed neurological and psychiatric deficits which remained unexplained despite a detailed work-up. The symptoms partly resolved after steroids implementation. At the last contact, 14 months after transplantation he remained in complete remission and his neurological condition improved substantially. Conclusions. Allo-SCT seems to be a promising treatment option for R/R HCL.","PeriodicalId":12837,"journal":{"name":"Haematologia","volume":"1 1","pages":"45-49"},"PeriodicalIF":0.0,"publicationDate":"2020-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88760851","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}

引用次数: 0

Leczenie nawrotowego/opornego szpiczaka plazmocytowego w Polsce w oparciu o program lekowy Ministerstwa Zdrowia w nawiązaniu do zaleceń Europejskiego Towarzystwa Onkologii Klinicznej

Haematologia Pub Date : 2020-06-30 DOI: 10.5603/hem.2020.0003

Grzegorz Charliński

引用次数: 0

Powikłania autologicznego przeszczepienia krwiotwórczych komórek macierzystych u pacjentki z układową amyloidozą AL

Haematologia Pub Date : 2020-06-30 DOI: 10.5603/hem.2020.0005

Agnieszka Końska, B. Pula, Tomasz Protaziuk, Joanna Sawczuk, Aleksander Salomon-Perzyński, A. Rydzewski, K. Jamroziak

引用次数: 1

Znaczenie pierwotnych niedoborów odporności ze szczególnym uwzględnieniem zmiennego pospolitego niedoboru odporności w rozwoju nowotworów limfoidalnych

Haematologia Pub Date : 2020-04-22 DOI: 10.5603/hem.2019.0033

Justyna Błach, Agnieszka Bojarska-Junak, J. Roliński

引用次数: 0

Różnice w obrazie niedokrwistości sierpowatokrwinkowej w zależności od czynników genetycznych

Haematologia Pub Date : 2020-04-22 DOI: 10.5603/hem.2019.0036

Henu Kumar Verma

引用次数: 0

Postępująca wieloogniskowa leukoencefalopatia u pacjenta z chłoniakiem rozlanym z dużych limfocytów B i zespołem nabytego niedoboru odporności

Haematologia Pub Date : 2020-04-22 DOI: 10.5603/hem.2019.0035

Katarzyna Brzeźniakiewicz-Janus, Agnieszka Lewosiuk, Zuzanna Walkowiak, Wojciech Pauli, G. Rutkowski, M. Parczewski, Mirosław Franków

引用次数: 0

Kompleks polimaltozy z wodorotlenkiem żelaza w leczeniu niedoboru żelaza i niedokrwistości z niedoboru żelaza w okresie ciąży

Haematologia Pub Date : 2020-01-01 DOI: 10.5603/HEM.2020.0041

Ibrahim A. Abdelazim, Mohamed M. Farghali, Osama O Amer

{"title":"Kompleks polimaltozy z wodorotlenkiem żelaza w leczeniu niedoboru żelaza i niedokrwistości z niedoboru żelaza w okresie ciąży","authors":"Ibrahim A. Abdelazim, Mohamed M. Farghali, Osama O Amer","doi":"10.5603/HEM.2020.0041","DOIUrl":"https://doi.org/10.5603/HEM.2020.0041","url":null,"abstract":"Introduction. Iron deficiency (ID), and iron deficiency anaemia (IDA) are consistently associated with reduced maternal cognitive function and increased depressive disorders. In addition, the preterm delivery, and intra-uterine growth restriction were reported as an adverse neonatal outcome for ID and IDA. This study designed to evaluate the efficacy of ferric hydroxide polymaltose (FPM) in treatment of ID, and IDA during pregnancy. Materials and methods . One hundred and twenty-two women with ID (ferritin < 15 μg/L), and moderate IDA (haemoglobin ≥ 7 and < 10 g/dL) during pregnancy were included in this study. Studied women treated with FPM tablets for ≥ 3 months. The pre-treatment ferritin, haemoglobin, red blood cells (RBCs)-mean corpuscular volume (MCV), and -mean corpuscular haemoglobin (MCH) were compared by post-treatment values. Results. The mean pre-treatment ferritin, and haemoglobin significantly increased from 12.4 ± 5.6 μg/L and 7.8 ± 3.3 g/dL; respectively to 116.5 ± 6.9 μg/L and 11.1 ± 2.8 g/dL; respectively, 3-months’ after FPM treatment (p = 0.02 and 0.0002; respectively). In addition, the mean pretreatment RBCs MCV, and MCH significantly increased from 73.5 ± 4.6 fL and 24.2 ± 7.7 pg; respectively to 94.0 ± 3.8 fL and 31.7 ± 6.3 pg; respectively 3-months’ after FPM treatment (p = 0.02 and 0.01; respectively). Conclusion. The FPM (Ferose ® ) is an effective therapeutic option for treatment of ID, and IDA during pregnancy with high safety profile, and low side effects. The superior tolerability of FPM is an important advantage because compliance to oral iron is the main obstacle toward effective treatment of ID, and IDA during pregnancy.","PeriodicalId":12837,"journal":{"name":"Haematologia","volume":"1987 1","pages":"212-218"},"PeriodicalIF":0.0,"publicationDate":"2020-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90364700","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}

引用次数: 0

Możliwość odstawienia nilotynibu u chorej na przewlekłą białaczkę szpikową w fazie głębokiej odpowiedzi molekularnej

Haematologia Pub Date : 2020-01-01 DOI: 10.5603/HEM.2020.0029

E. Wąsik-Szczepanek

引用次数: 0