Det myelodysplastiske syndrom I. Patogenese, klinik, diagnostik og differentialdiagnostik

Hans Carl Hasselbalch*, Birgitte Ravn Juhl, Per Boye Hansen

*Corresponding author af dette arbejde

    Publikation: Bidrag til tidsskriftReviewForskningpeer review

    Abstract

    The myelodysplastic (MDS) syndrome is characterized by variable cytopenia, owing to bone marrow insufficiency. Known provoking factors for the disease are chemicals (benzene), previous treatment with alkylating agents, and radioactive irradiation. The pathogenesis involves an acquired lesion of the pluripotent haematopoietic stem cell with the evolution of a (pre-) malignant cell clone with an increased proliferation potential, but, in addition, severe dysplasia with ineffective haematopoiesis. An increased intramedullary production of various cytokines that inhibit haematopoiesis, including tumour necrosis factor α (TNF-α), may be responsible for the accelerated cell death (apoptosis). An increasing genetic instability during the course of the disease causes progression of the cytopenia with anaemia, infections, and bleeding. Autoimmune diseases may be seen. The disease often progresses to acute myeloid leukaemia. A chromosomal analysis is important, as 40-50% of the patients have chromosomal changes at the time of diagnosis. Differential diagnostic considerations include temporary dysplasia provoked by medical or toxic agents, B12 or folate deficiency, infectious bone marrow involvement (HIV, CMV infection), chronic alcoholism, aplastic anaemia, and myelofibrosis.

    Bidragets oversatte titelThe myelodysplastic syndrome I. Pathogenesis, clinical symptoms, diagnosis, and differential diagnosis
    OriginalsprogDansk
    Sider (fra-til)476-479
    Antal sider4
    TidsskriftUgeskrift for laeger
    Vol/bind164
    Udgave nummer4
    StatusUdgivet - 21 jan. 2002

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