- Keywords
- tranzitorní abnormální myelopoéza,
- MeSH
- Down Syndrome * diagnosis complications MeSH
- Case Reports as Topic MeSH
- Leukocytosis blood physiopathology MeSH
- Humans MeSH
- Multiple Organ Failure * diagnosis etiology MeSH
- Myelopoiesis immunology MeSH
- Myeloproliferative Disorders diagnosis etiology classification MeSH
- Infant, Premature MeSH
- Infant, Newborn MeSH
- Check Tag
- Humans MeSH
- Infant, Newborn MeSH
- Publication type
- Review MeSH
- MeSH
- Anemia diagnosis drug therapy classification blood MeSH
- Blood Coagulation Disorders diagnosis etiology drug therapy classification MeSH
- Hemic and Lymphatic Diseases * diagnosis etiology drug therapy classification MeSH
- Blood Cell Count classification standards MeSH
- Lymphoproliferative Disorders diagnosis classification blood MeSH
- Myelodysplastic Syndromes diagnosis classification blood MeSH
- Myeloproliferative Disorders diagnosis classification blood MeSH
- Hemostatic Disorders diagnosis drug therapy classification blood MeSH
- Thrombophilia diagnosis etiology drug therapy classification MeSH
- Publication type
- Review MeSH
- MeSH
- Leukemia, Myelomonocytic, Chronic diagnosis pathology therapy MeSH
- Thrombocythemia, Essential diagnosis etiology therapy MeSH
- Humans MeSH
- Myeloproliferative Disorders * diagnosis etiology classification therapy MeSH
- Polycythemia Vera diagnosis etiology drug therapy MeSH
- Primary Myelofibrosis diagnosis etiology pathology therapy MeSH
- Check Tag
- Humans MeSH
- Publication type
- Review MeSH
- Keywords
- cytoredukční léčba,
- MeSH
- Anemia drug therapy MeSH
- Diagnosis, Differential MeSH
- Thrombocythemia, Essential diagnosis drug therapy therapy MeSH
- Humans MeSH
- Myeloproliferative Disorders * diagnosis drug therapy classification therapy MeSH
- Polycythemia Vera diagnosis drug therapy complications therapy MeSH
- Primary Myelofibrosis diagnosis complications therapy MeSH
- Prognosis MeSH
- Risk MeSH
- Splenomegaly drug therapy MeSH
- Treatment Outcome MeSH
- Check Tag
- Humans MeSH
- Publication type
- Review MeSH
Nejčastěji se vyskytující Ph negativní (Philadelphia chromosome‑negative) myeloproliferativní choroby (MPN) představují polycythaemia vera (PV), esenciální trombocytemie (ET) a primární myelofibróza (PMF). Objevy somatických mutací JAK2V617F, CALR a MPL se staly důležitými prvky pro pochopení patogeneze PMF a tyto mutace jsou odpovědné za vlastní fenotyp onemocnění. Ze skupiny inhibitorů JAK1/2 byl v roce 2011 prvním zástupcem ruxolitinib, který byl schválen americkým Úřadem pro kontrolu potravin a léčiv (FDA) pro léčbu PMF. Hematologická toxicita a zvýšené riziko infekcí jsou nejčastějšími nežádoucími účinky této léčby. I když se uskutečnily studie s dalšími inhibitory JAK1/2 (fedratinib, momelotinib, pacritinib), žádný další inhibitor do terapie MPN není dosud schválen.
The most common Philadelphia‑negative (Ph‑) myeloproliferative diseases (MPD) are polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF). The discoveries of JAK2V617F, CALR, and MPL somatic mutations have become important elements for understanding the pathogenesis of PMF, and these mutations are responsible for their own phenotype of the disease. In the JAK1/2 inhibitor group, ruxolitinib was the first drug to be approved by the Food and Drug Administration (FDA) for the treatment of PMF in 2011. The hematologic toxicity and increased risk of infections are the most common side effects of the treatment with ruxolitinib. Although studies with other JAK1/2 inhibitors (fedratinib, momelotinib, pacritinib) have been performed, no other ihibitors are approved for the therapy of MPD yet.
- MeSH
- Thrombocythemia, Essential drug therapy genetics MeSH
- Janus Kinase Inhibitors pharmacology therapeutic use MeSH
- TYK2 Kinase genetics metabolism drug effects MeSH
- Humans MeSH
- Myeloproliferative Disorders * drug therapy genetics classification MeSH
- Polycythemia Vera drug therapy genetics MeSH
- Primary Myelofibrosis drug therapy genetics MeSH
- Pyrazoles administration & dosage pharmacology adverse effects MeSH
- Risk Factors MeSH
- Check Tag
- Humans MeSH
- Keywords
- ruxolitinib,
- MeSH
- Humans MeSH
- Mutation MeSH
- Myeloproliferative Disorders diagnosis drug therapy genetics classification therapy MeSH
- Primary Myelofibrosis * diagnosis drug therapy genetics therapy MeSH
- Prognosis MeSH
- Pyrazoles therapeutic use MeSH
- Risk MeSH
- Risk Factors MeSH
- Splenectomy mortality adverse effects MeSH
- Statistics as Topic MeSH
- Stem Cell Transplantation mortality adverse effects MeSH
- Treatment Outcome MeSH
- Check Tag
- Humans MeSH
- MeSH
- Hematologic Neoplasms * diagnosis genetics classification therapy MeSH
- Humans MeSH
- Mutation * MeSH
- Myeloproliferative Disorders * diagnosis genetics classification therapy MeSH
- World Health Organization * MeSH
- Check Tag
- Humans MeSH
- Publication type
- Review MeSH
Kontinuálny rozvoj nových poznatkov v hematopatológii a najmä genetike hematologických nádorov si vyžiadal aktualizáciu široko akceptovanej a dodnes používanej WHO klasifikácie myeloidných neoplázií, publikovanej pre 8 rokmi. Základné princípy tejto klasifikácie však ostávajú nezmenené a preto autori novej WHO klasifikácie nehovoria o vzniku novej, ale skôr o „revízií“ pôvodnej klasifikácie. Cieľom tejto práce je priblížiť širokej verejnosti patológov, hematológov resp. iných klinikov v Českej Republike a na Slovensku najdôležitejšie zmeny v diagnostike myeloidných neoplázií, ktoré boli nedávno publikované mienkotvorcami v tejto oblasti.
Continual progress of knowleges in hematopathology and genetics of hematologic tumors requires actualisation of widely accepted and presently used WHO classification of myeloid neoplasms published 8 years ago. However, the basic principles of this classification remain unchanged, therefore the authors of new WHO classification mention the „revision“ of previous and not the introduction o new classification. The aim of this paper is to outline the most important changes of myeloid neoplasm diagnostics to pathologists, hematologists and other clinicians in Czech Republic and Slovakia. This review is based on several papers recently published by opinionleaders in this field.
- MeSH
- Thrombocythemia, Essential diagnosis MeSH
- Classification MeSH
- Myelodysplastic Syndromes diagnosis classification MeSH
- Leukemia, Myeloid diagnosis classification MeSH
- Myeloproliferative Disorders * diagnosis classification MeSH
- Polycythemia Vera diagnosis MeSH
- Primary Myelofibrosis diagnosis MeSH
- Publication type
- Review MeSH
- MeSH
- Anemia etiology drug therapy complications MeSH
- Hydroxyurea therapeutic use MeSH
- Janus Kinase Inhibitors therapeutic use MeSH
- Humans MeSH
- Myeloproliferative Disorders diagnosis classification therapy MeSH
- Primary Myelofibrosis * diagnosis classification therapy MeSH
- Splenectomy methods MeSH
- Splenomegaly etiology complications therapy MeSH
- Severity of Illness Index MeSH
- Hematopoietic Stem Cell Transplantation methods MeSH
- Bone Marrow Transplantation MeSH
- Check Tag
- Humans MeSH
- Publication type
- Review MeSH
