Chapter 16 in
haematology (Myelodysplasia)summary
-Myelodysplasia include a group of clonal disorders of heamopoietic stem cells that
lead to bone marrow failure and low blood cell counts.Ahallmark of the disease
is simultaneous proliferation and apoptosis is haemopoietic cells leading to
the paradox of a hypercellular bone marrow but pancytopenia in peripheral blood
. there is a tendency to progress to acute myeloid leukaemia.
-In most cases, the disease is primary but it may be secondary to chemotherapy given
for treatment of another malignancy.
-The main clinical features
of anaemia, infectionand bleeding, are caused by
reduction in the bone count.most patients are over 70 years of age.
-Diagnosis is made by
examination of the blood and bone marrow together with genetic studies of the
tumour cells . they are calssfiedinto eight major subtypes.
-Scoring systems can divide patients in those with low-grade or high-grade disease .
-Low-grade disease may not need treatemt . haemopoietic growth factors,lendalidomide or
blood product support may be usful when required.
-High-grade myelodysplasia may be treated by itensive chemptherapy,demethylating drugs or stem
cell transplantation.Allogeneic transplantation is the only curative procedure.
-Myelodusplasia/myeloproliferative
neoplasma are a group of disorders classified between myelodysplasia and
myeloproliferaive disorders and show the presence of dysplastic featurs but
also increased number of circulating .
From
book: Essentail Haematology A.V.HOFFBRAND,P.A.H.MOSS.6EDITION
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