Quiz part 2 C4 Flashcards
(11 cards)
define myelodysplastic syndrome aka myelodysplasia
a group of clonal disorders of multipotent haemapoietic stem cells, characterized by increasing bone marrow failure and ineffective haemotopoiesis and quantitative and qualitative of all 3 myeloid cells
what are the consequences of ineffective haemotopoiesis
cytopenia
what is the unique characteristics of MDS
hypercellular bone marrow but pancytopenia in PBF
why does MDS have hypercellular BM but pancytopenia in PBF
increased stem cell proliferation but ineffective differentiation and maturation
what are the clinical symptoms for MDS
- anaemia
- infections
- easy brusising/ bleeding
what are the incidence of MDS
- 4 in 100000
- male predominance
- common among elderly ( >50% are more than 70 yrs old)
- less than 25% are less than 50 yrs old
what does PBF shows in MDS
- pancytopenia
- macrocytic/ dimorphic/ hypochromic
- normoblast may be present
- low reticulocyte count
- low num of granulocytes and lack of granulation
- pelger abnormality
- myeloblasts may present
- low number of platelets
what are the characteristics of pelger abnormality
- inherited
- nuclei of wbc have unusual shapes
- bilobed, peanut or dumbell shaped
- indicates failure of neutrophil to segment properly
what are the lab finding of bone marrow
- multinucleated polychromatic normoblasts
- ring sideroblasts
- defect in [rimary and secondary granulation of granulocytes
- megakaryocytes with micronuclear, small binuclear or polynuclear forms
- dysplatic cells
what are the lab finding for cytogenetic tests
- partial or total loss of chromosome 5 or 7
- trisomy 8
- 5-q syndrome
[loss of chromosme 5 bands q 13 to q33] - RAS oncogene mutation
- FMS gene mutation
[ feline McDonough Sarcoma}
[ type III receptor tyrosine kinase ]
[ binds to macrophage or monocyte CSF (M-CSF/ CSF-1) ]
what are the classification of MDS
- blood count
- morphology
- number of blast cells in blood/ BM
