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  1. Article ; Online: Challenges of diagnosing and managing pre-fibrotic myelofibrosis: A case-based and practical approach.

    Rampotas, Alexandros / Hargreaves, Rupen / McLornan, Donal P

    Best practice & research. Clinical haematology

    2022  Volume 35, Issue 2, Page(s) 101378

    Abstract: Pre-Fibrotic Myelofibrosis is a frequently under-recognised entity that has distinct features separate to those of both Essential Thrombocythaemia and overt Primary Myelofibrosis. Misdiagnosis is relatively common due to subtle differences in bone marrow ...

    Abstract Pre-Fibrotic Myelofibrosis is a frequently under-recognised entity that has distinct features separate to those of both Essential Thrombocythaemia and overt Primary Myelofibrosis. Misdiagnosis is relatively common due to subtle differences in bone marrow trephine morphology and multidisciplinary approaches are required. The clinical phenotype and disease course is heterogeneous and hence management approaches tend to vary widely. Although patients may initially be asymptomatic, disease-related complications can include troublesome symptom burdens, increased incidence of both arterial and venous thromboses, haemorrhage, anaemia and an inherent risk of disease evolution to either overt myelofibrosis or blastic phase disease. Specific prognostic tools with high discriminatory power are lacking. Within this review we use case-based approaches to review the current literature, highlight challenges in both diagnostics and disease management and suggest contemporary approaches to improve patient outcomes.
    MeSH term(s) Humans ; Primary Myelofibrosis/diagnosis ; Primary Myelofibrosis/genetics ; Primary Myelofibrosis/therapy ; Thrombocythemia, Essential/diagnosis ; Thrombocythemia, Essential/genetics ; Thrombocythemia, Essential/therapy ; Bone Marrow ; Prognosis ; Disease Progression
    Language English
    Publishing date 2022-08-24
    Publishing country Netherlands
    Document type Journal Article ; Review
    ZDB-ID 2048027-1
    ISSN 1532-1924 ; 1521-6926
    ISSN (online) 1532-1924
    ISSN 1521-6926
    DOI 10.1016/j.beha.2022.101378
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Diagnostic and management strategies for Myeloproliferative Neoplasm-Unclassifiable (MPN-U): An international survey of contemporary practice.

    Hargreaves, Rupen / Harrison, Claire N / McLornan, Donal P

    Current research in translational medicine

    2022  Volume 70, Issue 3, Page(s) 103338

    Abstract: Myeloproliferative Neoplasm-Unclassifiable (MPN-U) is defined as an MPN that fails to meet the diagnostic criteria for any of the other defined classical or 'non-classical' MPNs. The reported incidence is variable, dependent on appropriate recognition, ... ...

    Abstract Myeloproliferative Neoplasm-Unclassifiable (MPN-U) is defined as an MPN that fails to meet the diagnostic criteria for any of the other defined classical or 'non-classical' MPNs. The reported incidence is variable, dependent on appropriate recognition, and the clinical course can be highly variable ranging from an indolent disorder through to an aggressive disease course with a significant risk of thrombosis, bulky splenomegaly, and debilitating symptom burden. Clinicians frequently manage these conditions according to the clinical concerns e.g. splenomegaly akin to the phenotype, but no evidence base exists. Currently, there are no widely accepted guidelines to deal with both the diagnostic work up and subsequent clinical management of this entity. We hence surveyed major International MPN centres to gain an understanding of common challenges in MPN-U management in 2021 and aid identification of considerable practice variations where harmonisation would be desirable. Such information is vital to support future consensus guidelines in addition to informing further collaborative studies.
    MeSH term(s) Humans ; Incidence ; Myeloproliferative Disorders/diagnosis ; Myeloproliferative Disorders/epidemiology ; Myeloproliferative Disorders/therapy ; Neoplasms ; Splenomegaly/diagnosis ; Splenomegaly/epidemiology ; Splenomegaly/etiology ; Surveys and Questionnaires
    Language English
    Publishing date 2022-02-23
    Publishing country France
    Document type Journal Article
    ISSN 2452-3186
    ISSN (online) 2452-3186
    DOI 10.1016/j.retram.2022.103338
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: How I manage myeloproliferative neoplasm-unclassifiable: Practical approaches for 2022 and beyond.

    McLornan, Donal P / Hargreaves, Rupen / Hernández-Boluda, Juan Carlos / Harrison, Claire N

    British journal of haematology

    2022  Volume 197, Issue 4, Page(s) 407–416

    Abstract: Myeloproliferative neoplasm (MPN)-unclassifiable (MPN-U) or not otherwise specified represents a rare, poorly defined and heterogeneous group of MPNs. Disease incidence is difficult to define but likely represents close to 5% of all MPNs when strict ... ...

    Abstract Myeloproliferative neoplasm (MPN)-unclassifiable (MPN-U) or not otherwise specified represents a rare, poorly defined and heterogeneous group of MPNs. Disease incidence is difficult to define but likely represents close to 5% of all MPNs when strict World Health Organisation (WHO) criteria are applied. Dynamic review over time is required to assess if the disease can be re-classified into another MPN entity. A diagnosis of MPN-U leads to many challenges for both the patient and physician alike including lack of agreed monitoring and therapeutic guidelines, validated prognostic markers and licenced therapies coupled with exclusion from clinical trials. MPN-U has an inherent risk of an aggressive clinical course and transformation in some but who, and when to treat in the chronic phase, including identifying who may require more aggressive therapy at an earlier stage, remains elusive. Moreover, despite the significant thrombotic risk, there is no agreement on systematic primary thromboprophylaxis. We hereby provide a contemporary overview of MPN-U in addition to four illustrative cases providing our collective suggested approaches to clinical challenges.
    MeSH term(s) Anticoagulants ; Humans ; Myeloproliferative Disorders/diagnosis ; Myeloproliferative Disorders/therapy ; Neoplasms ; Venous Thromboembolism
    Chemical Substances Anticoagulants
    Language English
    Publishing date 2022-02-22
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 80077-6
    ISSN 1365-2141 ; 0007-1048
    ISSN (online) 1365-2141
    ISSN 0007-1048
    DOI 10.1111/bjh.18087
    Database MEDical Literature Analysis and Retrieval System OnLINE

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