quiz Flashcards
Fanconi
DNA repair and damage, 12 different genes
Shwachman Diamond
same gene, ribosomal RNA, mitotic spindle
dyskeratosis
telomerase function abnormally short, Xlinked
congenital amegakaryocytic thrombocytopenia
thrombocytopenia and absent kegakaryocytes, mutations in MPL
paroxysmal nocturnal hemoglobinuria
hemolysis and BM failure, less GPI anchor on RBC, complement lysis, thrombosis
toxic granulation
dark granules in neutrophils seen with infection or G-CSF
Dohle bodies
ribosome rich ER seen in sepsis, G-CSF use
Pelger-Huet
bilobed nucleus, myelodysplastic syndrome
anemia
decrease in hemoglobin and hemotocrit
MCV
size of RBC
MCH
hemoglobin concentration
MCHC
hemoglobin concentration in volume of red cells
RDW
red cell distribution
reticulocyte
pre red cell
retic count
percentage of retics in RBC
reticulocyte index
retic count X Hct / 40
should be greater than 2 in anemic patient with good response
hypo vs hyper proliferative depends on…
reticulocyte index or absolute retic count
tranferrin
iron transport in blood
hepcidin
prevents iron release, prevents absorption, prevents iron export
serum ferritin
relfect iron stores
labs in iron def
low MCV
low MCH
decreased RBC
low retic
serum iron studies in Fe def
low serum Fe
High TIBC - transferrin can bind
Ferritin - low
transferritin sat - low
difference in anemia of chronic disease in iron test
decreased TIBC and increased or normal ferritin
hereditary spherocytosis
defects in spectrin, ankyrin, band 3 and pallidin, memebrane deformability
