Hematology 4 Flashcards
hereditary spherocytosis cause
Ankyrin
Hereditary elliptocytosis cause
Spectrin A
Southeast Asian ovalocytosis cuase
Band 3
Beta globin chain located on Ch
11
Hb Lepore
delta-beta fusion
MCV/RBC ratio in thalassemia
<13
HbConstant Spring
extra-long alpha chain (looks like alpha thal)
HbH disease
deletion of 3 alpha genes, Beta tetramers
%HbS in SA
35-45%
%HbS in S-alpha-thal
23-35%
% HbS in S-Beta-thal
> 80%
Most specific (1) and most sensitive (1) antibodies in pernicous anemia
specific = anti-intrinsic factor sensitive = anti-parietal cell
homocysteine and methylmalonic acid both increased in
B12 deficiency
Defect in Pelger Huet
LaminB mutation
Chronic granulomatous disease infections
catalast + bacteria, fungal, S aureus, salmonella, serratia, aspergillus, nocardia
MCV in alpha-thal
65-75
MCV in beta-thal
55-65
complications more common in SC dz than SS
avascular necrosis and proliferative retinopathy
HbC mutation
Glu6Lys
HbE mutation
Glu26Lys
HbG is ___ gene defect
alpha
Hb Lepore on elecrophoresis
looks like 15-30% HbS
Hbs with increased oxygen affinity
Chesapeake, Denver (show erythrocytosis)
Unstable Hbs
Hasharon, Koln, Zurich, Hammersmith (most severe)
only method that detects methemoglobin
co-oximeter
location of alpha chain gene
CH16
location of beta chain gene
Ch11
most common alpha thal genotype in african americans
-/alpha
Alpha thal2
alpha+thal
