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
Hexose 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-CV reference range
11.5% to 14.5%
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
MCV and RDW in sickle cell anemia
Normal MCV and high RDW
MCV and RDW in liver disease
High MCV and Normal RDW
MCV and RDW in megaloblastic anemia
High MCV High RDW
General term for a variation in the normal coloration
Anisochromia
Normally, RBCs have a central area of pillow approximately ____ the diameter
1/3
May also mean the occurrence of hypohromic cells and normochromic cells in the same blood smear
Anisochromia
May be found in Sideroblastic anemias, also in a hypochromic anemia after transfusion with normal cells and some weeks after iron therapy for IDA
ANISCHROMIA
Central pallor >1/3 of diameter
Hypochromic
Grading : central pallor 1/2
1+
Grading : central pallor 2/3
2+
Grading : central pallor 3/4
3+
Grading : 4+
Thin rim of hemoglobin
Anulocyte is also known as
Pessary cell, ghost cell
Rbc with a thin rim of hemoglobin and a large, clear center ; may be observed in IDA
Anulocyte
RBCs that lack central pallor even through they lie in desirable area for evaluation
Hyperchromic cells
Basically the only disease in which MCHC is high
Hereditary spherocytosis
3 clinical manifestations of Hereditary spherocytosis
Splenomegaly
Anemia
Jaundice
Immune disorders with spherocytes result on the DAT
POSITIVE
TEST INCREASED IN HEREDITARY SPHEROCHYTOSIS
MCHC (slightly)
AUTOHEMOLYSIS (greatly)
OFT (
Result of fluorescence in EMA binding test for HS
Low
Larger than normal red cell with bluish tinge in wright stain
Polychromatophilic erythrocytes
Bluish tinge in retics is caused by the presence of
Residual RNA
POLYCHROMASIA GRADING: 1%
Slight
POLYCHROMASIA GRADING: 3%
1+
POLYCHROMASIA GRADING: 5%
2+
POLYCHROMASIA GRADING: 10%
3+
POLYCHROMASIA GRADING: >11%
4+
Increased number of red cells with variation in SHAPE
POIKILOCYTES
Almost spherical in shape ; lacks central pallor
Spherocytes
Appearance of rbc in the feathered edge
Lack central pallor
It can also result to spherocytosis red cells
Natural cell death
Aka “mouth cell”
Stomatocytes
Elongated rbcs with a slit like central pallor
Stomatocytes
Rh deficiency , alcoholism, severe liver disease, Overhydrated and DEHYDRATED hereditary ____
Stomatocytes
A dehydrated form of a stomatocyte
Xerocyte
Appears to have puddled at one end (half-light, half dark)
Xerocyte
Aka “thorn cell or spur cell”
Acanthocytes
Rbcs with irregularly SPICULATED surface
Acanthocytes
Abetalipoproteinemia is associated with what type of red cells shape
Acanthocytes
Abetalipoproteinemia is also known as
Basset-kornzweig syndrome or hereditary acanthocytosis
Characterized by defective apo B synthesis ; VLDL, LDL, and chylomicron not found in plasma
Abetalipoproteinemia
Cells seen McLeo syndrome and PK deficiency
Acanthocytes
Aka “echinocytes”
Burr cell
RBs with REGULAR SPICULES
Burr cell
Rbc seen in uremia and PK deficiency
Burr cell/ echinocyte
Cigar shaped rbc
Elliptocyte
Thalassemia is associated with what rbc
Elliptocyte
Aka “Mediterranean anemia, hereditary leptocytosis”
Thalassemia
Aka “teardrop cell”
Dacryocyte
Pear shaped rbc
Dacryocte
Rbc seen in primary myelofibrosis and megaloblastic anemia
Dacryocyte
Aka “schizocytes”
Schistocytes
Fragmented rbcs
Schistocyte
MAHAs are associated with what rbc shape
Schistocytes (helmet cells)
Group of disorders characterized by RBC fragmentation and thrombocytopenia
MAHAs
Major MAHAs
Thrombocytopenia purpura
HELLP (hemolysis, elevated liver enzymes, low plt ct)
Hemolytic uremic syndrome
Dessimenated intravascular coagulation
Characterized by narrowing obstruction of small blood vessels by fibrin or platelet aggregates resulting in rbc fragmentation
MAHAs
Also known as defibrination syndrome, consumption coagulopathy
DIC
Generalized over-activation of the coagulation and fibrinolytic systems
DIC
TESTS for DIC
D-dimer
Prothrombin fragment 1 + 2
Related conditions to DIC
TISSUE TRAUMA
OBSTETRIC COMPLICATIONS
MUCUS-SECRETING TUMORS
ACUTE INFECTIONS
SNAKE BITES
ACUTE PROMYELOCYTIC LEUKEMIA
AKA sickle cell ; meniscocyte
Drepanocyte
Two forms of drepanocytes
Irreversible sickle cell
Oat shaped cells
Crescent shaped ; with long projection ; when deoxygenated they fragment
Irreversible sickle cell
Have less pronounced projections ; when reoxygenated they return to the original biconcave disks
Oat shaped cells
Aka codocyte, platycyte, Greek helmet cell, Mexican hat cell, bull’s eye cell, leptocyte
Target cell
Rbc seen in LCAT deficiency
Target cell
Rbcs with an increased cell membrane-to-volume ratio
Codocytes
In vivo and on electro microscopy, these cells appear bell shaped
CODOCYTES
AKA DEGMACYTE
BITE CELL
Demonstrate a semicircular defect in their edge (resembles a bite mark)
Bite cell
Heinz bodies are seen in this rbc
Bite cell
Rbcs seen in G6PD deficiency
Bite cell
Folded rbcs
Biscuit cell
Bipolar or central distribution of hgb
Bronze Elliptocyte
As large a leukocytes ; pale pink staining ghost of the red cell
Semilunar body
Frequently seen in malaria and other conditions causing overt hemolysis
Semilunar bodies
Aka punctuate basophilia
Basophilic stippling
Associated with lead poisoning , megaloblastic anemia, arsenic poisoning , pyrimidine-5 nucleotidase deficiency, thalassemia
Basophilic stippling
A basophilic stippling in which PLUMBISM may be observed , megaloblastic anemia , impaired Hb synthesis
Coarse stippling
Aka papenheimer bodies
Siderotic granules
Iron stains like Perl’s reagent through Prussian blue reaction stains what
Siderotic granules
New methylene and wrights stains stain what rbc inclusion
Papenheimer bodies
Characterized by a DIMORPHIC peripheral blood picture and hypochromic RBCs in the same blood smear
Sideroblastic Anemia
Described as multiple dark blue irregular granules in Prussian blue iron staining
Siderotic granules
Described as pale blue clusters in wrights staining
Siderotic granules
Frequently appears single in a cell usually round <1 um diameter blue to purple in color
Howell Jolly bodies
Remnants of nuclear chromatin (DNA)
Howell-Jolly bodies
Fuelgen reaction of Howell jolly bodies
Positive
Described as threadlike structures that appear as purple blue loops or rings
Cabot rings
Mitotic spindle Remnants
Cabot rings
Most of the time appear eccentrically along the inner RBC membrane large. Round blue to purple materials
Heinz bodies
Denatured and precipitated hemoglobin
Heinz bodies
Supravital stains used for staining Heinz bodies
Brilliant cresyl blue
New methylene blue
Methyl blue
Crystal violet
Inclusion bodies seen in G6PD deficiency (FAVISM), Drug induced hemolytic anemia
Heinz bodies
Described as SMALL , multiple, evenly distributed throughout the red cell, granular, GREENISH BLUE bodies
He H inclusion bodies e
An abnormal Hb composed of 4 beta globin chains
Hemoglobin H
A subtype of alpha thalassemia
Hb H disease
Schuffner dots is seen what protozoan
Malaria
Aka eosinophilia stippling
Schuffner dots
“Maltese cross”
Babesia spp.
Caused by a serum protein abnormality either increased globulin or fibrinogen
Rouleaux formation
Many be observed in multiple myeloma
Rouleaux
These are graded as POSITIVE ONLY
Sickle cells
Basophilic stippling
Papenheimer bodies
Howell Jolly bodies
Erythrocyte morphology grading : Slightly increased or few
1+
Erythrocyte morphology grading : moderately increased
2+
Erythrocyte morphology grading : Many
3+
Erythrocyte morphology grading : marked or marked increase
4+
