Flashcards in Heme Deck (367)
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1
Normoblasts
Nucleated RBCs
2
Reticulocytes
When nucleated RBCs lose their nucleus (5-7 days)
3
What causes left shift of O2 binding
Increase pH
Decrease DPG/Temp
4
What causes right shift of O2
Decreased pH
Increased DPG/TEMP
5
What is the major Hemoglobin structure for adults
HbA (alpha/beta) 95%
6
Why are RBCs binconcave in shape
Enables max O2 saturation
Allows passage through small capillaries and splenic sinusoids
7
Anemia
Lower than normal total circulating red cell mass
8
Mean cell volume
MCV
Volume of RBC
Femtoliters
9
Mean cell hemoglobin
MCH
Average content of Hb
Picograms
10
Mean cell hemoglobin concentration
MCHC
Concentration of Hb in given packed RBCs
11
Red cell distribution
RDW
Coefficient of variation of RBC volume
12
Anemia due to acute hemorrhage, what is the immediate concern
Hypovolemia
13
Are the majority of hemolytic anemias found intravascular or extravascular
Extravascular
14
Intravascular hemolysis Causes
Complement fixation: BIGGEST! think of blood mismatching
Mechanical injury
Infections/ intracell parasites, toxins
15
What happens to RBCs in extravascualr hemolysis
RBCs rendered less deformable
RBCs thought as foreign
16
Lab: PB- normochromic, normocytic anemia, increased reticulocytes
BM- erythroid hyperplasia
Serum: Increased unconjugated bili, increase LDH, low haptoglobin
Hemolytic anemia
17
Urine: hemosiderinuria, +/- hemoglobinuria
Intravascular hemolysis
18
Hereditary intrinsic RBC defects
Spherocytosis: membrane defect
G6PD: Enzyme defect
Sickle: hemoglobin defect
19
Acquired extrinsic RBC defects
Immune mediated damage
Nonimmune mediated damage
Sequestration- hypersplenism
20
Who has highest incidence of spherocytosis
N. Europeans
21
What is the genetic pattern for spherocytosis
AD
22
What is the lifespan of RBCs in spherocytosis
10-20 days
23
What are the proteins affected in spherocytosis
Ankyrin (75%)
Band (20%)
Ankyrin, spectrin, band 4.1, band 4.2
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Why is there splenomegaly in spherocytosis
Deformed RBC (more spherical) unable to traverse splenic sinusoids, phago by splenic macrophages
25
Tx of spherocytosis
Splenectomy
26
mild to chronic hemolytic anemia
Induced aplastic/ hemolytic crisis
hereditary spherocytosis
27
What is the one test for spherocytosis
Osmotic fragility test
In 65% of patients RBCs lyse prematurely when exposed to hypotonic salt solutions
28
G6PD is in which cycle
Hexose monophosphate shunt (glutathione metabolism)
29
What are the G6PD variants which cause disease
A: 10% africans
Mediteranian: mostly middle east
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