ERYTHROCYTES & ANOMALIES Flashcards
The RBC count, hemoglobin, and hematocrit values are _____ in people living a ______
Elevated ; higher altitude
Normal rbc count in children
4.00 to 5.40 x 10^6/uL or x 10^12/L
Adult male normal rbc count
4.60 to 6.00 x 10^6/uL or x 10^12/L
Adult female rbc count normal
4.00 to 5.40 x 10^6/uL or x 10^12/L
Glucose penetrates the red blood cell with no energy expenditure via _______(transmembrane protein)
Glut-1
This pathway handles 90% of glucose utilization in red blood cells
Non oxidative, anaerobic pathway that produces 2 molecules of ATP
EMBDEN MEYERHOF PATHWAY
An autosomal recessive disorder ; most common enzyme deficiency of he EMP and most common form of hereditary nonspherocytic hemolytic anemia
Pyruvate kinase deficiency
Possible cells seen in PBS of PK deficiency
Acanthocytes, burr cells
Recommended screening test for PK deficiency
PK fluorescent spot
Also a screening test for PK deficiency
Auto hemolysis test
A confirmatory test for PK deficiency
Quantitative PK assay
Pattern of autohemolysis with PK deficiency : it is greatly ______ and glucose has _____
INCREASED ; NO EFFECT
These are the three alternate pathways that branch from the glycolytic pathway
Glycolysis diversion pathways (Shunt)
Pentose phosphate pathway to phosphogluconate pathway
Heroes monophosphate shunt (Aerobic glycolysis)
This pathway prevents the desaturation of the GLOBIN by oxidation
Hexose monophosphate shunt
Functionally dependent on G6PD
HEXOSE MONOPHOSPHATE SHUNT
An x-linked recessive disorder ; most common deficiency in the pentose phosphate pathway a
G6PD DEFICIENCY
Most common RBC enzyme defect
G6PD deficiency
An autosomal recessive disorder in the embden-Meyerhof pathway
Pk deficiency
An x-linked recessive disorder in the Hexose monophosphate shunt
G6PD deficiency
Cells seen in the PBS of a ptx with G6PD deficiency
Heinz bodies and bite cells
Recommended screening test for G6PD deficiency
G6PD fluorescent spot test
Also a screening test for G6PD deficiency
Autohemolysis test
Confirmatory test for G6PD
Quantitative G6PD assay
Chronic type of G6PD , hereditary nonspherocytic henmolytic anemia
Type 1
A severe type of G6PD, episodic acute hemolytic anemia associated with infections, certain drugs, and fava beans ; not self limited and may require transfusion during hemolytic episode
Type 2
Favism is mostly demonstrated in what type of G6PD deficiency
G6PD-Mediterranean
Episodic type of G6PD deficiency, acute hemolytic anemia associated with infections and certain drugs; self limited
Type III
G6PD-Serres
G6PD -Madrid
Type I
G6PD-Mediterranean
G6PD-Chatham
Type II
G6PD-A”
G6PD-Canton
Type III
G6PD-B (Wild type)
GPD-A= (May also manifest as class III)
Type IV
Most frequently encountered enzymopathies are deficiencies of
G6PD and PK
AKA cytochrome B5 reeducase
Methemoglobin reductase
For the production of 2,3-BPG
Rapoport-Luebering shunt
It binds to the hemoglobin and decreases the oxygen affinity of hemoglobin
2,3-BPG
Two variables affecting the degree of association or dissociation between oxygen and hemoglobin
Partial pressure of oxygen
Affinity of hemoglobin for oxygen
Affinity of hemoglobin for oxygen is depended on 5 factors
pH
Partial pressure of CO2
Concentration of 2,3-BPG
Temperature
Presence of other hgb species that are nonfunctional
The curve produced when the 2 variables (partial pressure of O2 and affinity of hgb for O2) are plotted in a graph (oxygen saturation of hgb vs partial pressure of O2)
Oxygen dissociation curve
OXYGEN DISSOCIATION CURVE: Shift to the left
⬆️ pH
⬇️ PCO2
⬇️2,3-BPG
⬇️TEMPERATURE
INCRESEAD AFFINITY
OXYGEN DISSOCIATION CURVE: Shift to the RIGHT
⬇️ pH
⬆️ PCO2
⬇️ 2,3-BPG
⬇️ Temperature
Decreased affinity
A shift in the curve due to an alteration in pH
Bohr effect
Depict the occurrence by which the binding o2 to the hgb promotes the release of CO2
Haldane effect
Increased number of red cells with variation in size
Anisocytosis
Sized of RBC usually seen when MCV is 80-100 fL
6-8 um
Anisocytosis :
> 8.0 um
100 fl
Macrocytosis
Anisocytosis:
<6.0 um
<80 fL
Microcytosis
MCV formula
HCT/RBC CT X 10
A visual display of cell size (x-axis) and cell friquency is the number of cells (y-axis)
Blood cell histogram
Automated hematology analyzers produce histograms for
Rbcs, WBCs, and platelets
Automated hematology analyzers produce histograms for
Rbcs, WBCs, and platelets
Two parameters calculated from RBC histogram
MCV and RDW
If The rbcs are macrocytic, the curve will shift to the
Right
If the rbcs are microcytic , the curve will shit to the
Left
Shape of the histogram curve
Bimodal
Conditions that may cause a bimodal curve
Blood transfusion
Cold agglutinin disease
Hemolytic anemia with schistocytes present
Indicates more variation in the size of the cells
A wide or flattened curve on histogram
A calculated index given by hematology analyzers to help identify Anisocytosis and provide information about its degree
RDW
Based on both of the RBC distribution curve and the mean RBC size
RDW-CV
Based on the actual measurement of the width of the RBC distribution curve in fĻ
RDW-SD
RDW-CF reference range
11.5% to 14.6%
RDW-SD reference range
39-46 fL
EARLIEST METHOD provided by the hematology analyzers to measure the red cell variations
RDW-CV
Dependent on the width of the distribution curve and the MCV
RDW-CV
Width of the curve is measured at the point that is 20% above the baseline
RDW-SD
Better and more reliable measure of erythrocyte variability, specifically in highly abnormal conditions
RDW-SD
MCV and RDW in microcytic
Low MCV and high RDW
RDW Reference range for newborns
14.2 to 19.9%
RDW is markedly _____ in newborns but gradually, the value will ____ until it reaches adult levels by _____ (age)
Incresead ; decrease ; 6 months of age
RDW and MCV in Anemia of chronic inflammation
Low MCV and Normal RDW
MCV and RDW in iron deficiency anemia
Low MCV and high RDW
MCV and RDW IN G6PD deficiency
Normal MCV and normal RDW