RBCs
________ is a condition where RBCs cannot supply O2 to the bodys tissues
anemia
Anemias are classified ___________ and _____________
morphologically and physiologically
Microcytic/Hypochromic anemia develops, causing an MCV < __, MCHC < __, and hemoglobin deficiency
80; 32
Symptoms of microcytic/hypochromic anemia are _____, _____, _____ ______, _______, and _____ ______
Fatigue, Dypsea, Angina pectoris, syncope, and heart problems
Iron is in multiple forms, such as _____________________
heme, nonheme, ferritin, and hemosiderin
Iron and globin is recycled as a result of ___ ___________
RBC senescence
Iron absorption happens through the ___ ____
GI tract
Infants recycle ___% of iron used for RBC production
70
Adults recycle ___% of iron used for RBC production
95
Children should absorb ~ ___mg/day of iron
0.5
women should absorb ~ ___ - ___mg/day of iron
0.2-2
Men and infants should absorb ~ ___mg/day of iron
1
IDA stage __ is the continuous depletion of iron from the bone marrow
1
IDA stage __ is the presence of slight microcytic, hypochromic pictures
2
IDA stage __ is the presence of microcytes and hypochromia
3
IDA _______________ is changes in body functions related to iron deficiency
pathophysiology
Unique symptoms of IDA is ____, ________, and ________
Pica, cheilitis, and Koilonychia
As iron decreases, _____ increases
TIBC
Serum iron should be ______ ug/dL
50-150
Serum Ferritin should be ______ ug/L in males and ______ ug/L in females
15-250; 10-120
The TIBC should be ________ ug/dL
250-400
Transferrin saturation should be _____%
20-55
_________ ______ are the accumulation of iron in the mitochondria
Sideroblastic anemias
Sideroblastic anemias are aquired as a result of ____ ________
hgb transfusion
With sideroblastic anemias, _____________ _________ and _________ _____________ are present
Pappenheimer bodies and ringed sideroblasts
____________ _______________ is caused by an abnormal gene called HFE
Hereditory Hemochromatosis
_____ causes iron to not bind with transferrin and be placed into storage continuosly
HFE
HH is confirmed with a serum ferritin level >_____uq/L and transferrin saturation >___%
300; 60
___________ is defects in hemoglobin a or b globin chain synthesis
Thalassemia
Thalassemia has no relation to ____ levels or diseases
iron
______ ___________ results in problems in specifically alpha globin chain deletion
Alpha Thalassemia
______ ________ _______ is the complete absence of alpha globin chains in fetal babies, most of which are immediately aborted
Bart's Hydrops fetalis
____________ __ _______ is where hgb H is formed instead of hgb A, usually with only 1 A chain and 3 H chains
Hemoglobin H disease
Hemoglobin H disease has golf ball like ___
RBC
____ _________ ____ is present with two functional A globin chains
Alpha Thalassemia Trait
______ ______ is the presence of 3 functional alpha genes
Silent carrier
________ ___________ _______ is anemia with little to no functional Beta globin chains being synthesized
Beta Thalassemia Major
Beta Thalassemia Major can be treated with __________________________
transfusion, splenectomy, bone marrow transplant, and stem cell transplant
_______ __________ _________ develops later in life
Beta Thalassemia intermedia
______ ___________ _____ (minor) has one abnormal B gene that is inherited
Beta Thalassemia trait
__________ _________ is an anemia cause by Vit B12 and Folate deficiency which causes impaired DNA synthesis
Megaloblastic Anemia
_________ ______ (IF) binds and transports Vit B12 through the stomach
Intrinsic Factor
Megaloblastic anemia causes issues with RBC maturation, such as __________ ______________ and _____________ ___________
Ineffective Erythropoiesis and Asynchronous Maturation
__________ __________ causes the nucleus to develop slower and be smaller than the cytoplasm
Asynchronous maturation
_____________ ____________ causes a lack of nRBCs and reticulocytes in the peripheral smear
Ineffective Electropoiesis
____________________ is a lack of reticulocytes
Reticulocytopenia
__________ _________ is caused by the lack of IF, decreasing Vit B12 absorption
Pernicious anemia
Vit B12 is tested using _______________
Radioimmunoassays
Folate is measured using ______ _____
Serum Tests
MMA and homocysteine are increased with _______ deficiency
Vit B12
Non megaloblastic anemia symptoms are ________________________________________
round hypochromic macrocytes caused by alcoholism, hypothyroidism, and liver disease
Vit B12 and Folate deficiency causes megaloblastic anemia, as well as __________________
reticulocytopenia
Symptoms of megaloblastic anemia are ___________________________________________
Hypersegmented Neutrophils, Macro Ovalocytes, Ineffective Erythropoiesis, and Demyelination
Hereditary Spherocytosis is common among ______________________
Northern Europeans
__________ __________ is a defect with a deficiency in membrane proteins spectrin and ankyrin (Protein band 3 and 4.2)
Hereditary Spherocytosis
Spherocytes are formed in the _________
spleen
_________ _______________ is a red cell membrane deficiency of the protein stomatin
Hereditary Stomatocytosis
______________ have increased Na+ and H2O, causing RBCs to swell
Stomatocytes
Stomatocytes have decreased _________ and increased ____
MCHC; MCV
_____________ ______________ is where RBCs membrane (A and B regions specifically) has defective or deficient spectrin
Hereditary Elliptocytosis
Hereditary Elliptocytosis has 4 subtypes, ____________________________________________
common hereditory elliptocytosis, Southeast Asian Ovalocytosis, Spherecytic hereditory elliptocytosis, and hereditary pyropoikilocytosis
Common hereditary Elliptocytosis is either present as mild or severe, the ______ version however is only in infants
severe
Spherocytic hereditory elliptocytosis is both _______________ and _________________ present; also associated with gall bladder disease and splenomegaly
spherocytes and elliptocytes
____________ _____ _________________ is present with a band 3 defect, also provides mild protection against malaria
Southeast Asia Ovalocytosis
_____________ ____________________has red cell budding, rare elliptocytes, and spherocytes in circulation
Hereditary pyropoikilocytosis
_____________ ____________ _______________ causes ATP to not form from ADP, making Burr cells present
Pyruvate Kinase deficiency
Pyruvate kinase if screened using a ______________ screening test followed by a specific assay
fluorescent
________ is a key part of the phosphogluconate pathway by keeping glutathione in a reduced state in order to reduce oxidative stress
G6PD
_________ ______ leads to hemolysis
Oxidative stress
___________ __________ ________ offers protection from malaria
X-linked recessive disorder
_____________ ____ ________ ___________ occurs within 24 hours of ingestion/injection of a drug
Drug-induced acute hemolytic anemia
________ occurs from contact with Fava Beans
Favism
G6PD has 4 clinical conditions, ______________________________________
Drug-induced acute hemolytic anemia, favism, Neonatal Jaundice, and Congestional Nonspherocytic Hemolytic Anemia
________________ ____________ occurs 2-3 days after birth
Neonatal Jaundice
Timing of G6PD testing is crucial and cannot be preformed during a _________ _________
Hemolytic episode
Hemoglobinopathies are disorders related to hemoglobin caused by ____________, ________ __________, _______, and ___________ of the globin chains
substitution, abnormal synthesis, fusion, and extension
If you have Hemoglobin S, _______ replaces glutamic acid on the 6th position of B chains
valine
Sickle cell anemia is inherited ___________ _______________
Autosomal Codominantly
Sickle cell ______ comes from when one of two parents pass sickle cell (Heterozygous)
trait
Sickle cell ______ comes from when both parents pass sickle cell (Homozygous)
Disease
Hgb S forms sickle cells in areas of _____ oxygenation
Low
__________ decreases RBC life span to 10-20 days
Sickling
Sickle cell cannot be diagnosed until the age of __+ months due to adult hgb not forming until then
6
_____________ (gall bladder stones) are present with Sickle Cell Disease
Cholelithiases
The presence of ____ suggests Spleen dysfunction
HJBs
_________, _____ _________, and ________ ____________ are all physiological conditions of Sickle cell Disease
Anemias, lung conditions, and Spleen conditions
Vaso-Occlusive episode trademark symptoms are ______ _________, ____________, ______________, and ___________
painful crisis', priapism, Retinopathy, and Strokes
The severity of Sickle cell depend on the ____________
Haplotype
The _________ Haplotype is located in Saudi Arabia and has a Hgb F % of greater than 20%
Asian
The _________ Haplotype is located in the West Africa Coast and has a Hgb F % of 5-20%
Senegal
The ________ haplotype is located in West Africa and has a Hgb F % of <10%
Benin
The ________ haplotype is located in Central south Africa and has a Hgb F % of 5-20%
Bantu
The ________ Haplotype is located in the Caribbean and has a Hgb F % of less than 5%
CAR
_____________ increases Hgb F which inhibits sickling
Hydroxyurea
Treatment of sickle cell anemia is the use of ________________________________________________________
prophyulactic antibiotics, transfusion over 3-5 weeks, and hydroxyurea
With the formation of Hgb C, ______ replaces glutamic acid on the 6th position of the B chain
lysine
Sickle Cell Disease has a Hgb S presence of >__%
80
____________ sickle cells have a blunted end
Reversible
___________ sickle cells have a pointed end
Irreversible
Sickle cell trait has a Hgb S concentration of ~__%
40
Hemoglobin C Disease has a Hgb C presence of >__%
80
The hallmark sign of hemoglibin C disease is its ________________
Crystals which resemble bars of gold
Hemoglobin C trait has a Hgb c presence of ~__%
40
_____________ __ disease has a 50% concentration of both hemoglobin S and C
Hemoglobin SC
The hallmark sign of hemoglibin SC disease is its ________________
crystals which resemble the washington monument and a gloved hand
Hemoglobin E has lysine replace glutamic acid on the __th position
26
Hemoglibin E disease has a Hgb E concentration of 30% with a _____________________ picture
Microcytic/hypochromic
Hemoglobin __ and __ migrate to the same place as Hgb S at a pH of 8.6 alkaline electrophoresis
D and G
Hemoglobin __ is isolated with a citrate electrophoresis at pH 6.0
O
Hemoglin S-beta Thalassemia are _______________________ and has no Hgb A present
microcytic/hypochromic
____________ _______________ isolates Hgb bands, which is quantified using densitometry
Hemoglobin electrophoresis
___________ ___________ uses a polyacrylamide gel at a pH of 3-10 and is used primarily for newborn screening
Isoelectric Focusing
Electrophoresis allows Hgb identification through _________ _________ and different media types
migration patterns
__________________ is the percentage of total blood volume occupied by erythrocytes
Microhematicrit
Microhematicrit is used to monitor __________ and ____________ conditions as well as evalute fluid status
Anemias and hemorrhage
____________ is determined by spinning a whole blood filled capillary tube in a microhematicrit centrifuge
Hematicrit
The hematicrit reference range in males is _____%, females _____%, and newborns ______%
42-52, 37-47, 53-65
____ is a nonspecific screening test for inflammation
ESR
Inflammation causes RBCs to fall __________
quicker
With ESR, add EDTA blood to the testing tube and stand _________ and _______________
vertically and undisturbed
ESR tests are __________
nonspecific
Under 50 the normal ESR range in males is _____mm/hr and females _____mm/hr
0-15, 0-20
Over 50 the normal ESR range in males is _____mm/hr and females _____mm/hr
0-20, 0-30
The most common factor affecting ESR testing is incorrect ______ __________________
tube fillage
The _____________ ________ is the most cost effective monitor of erythropoiesis
Reticulocyte count
% retics = # retics x ___/____
100/1000
Adults retic ranges are _______%
0.5-2
Infants retic ranges are ____%
2-6
Reticulocyte count is increased by __________
Hemolytic anemias, hemorrhage, iron therapy, uremia, and medications
Reticulocyte count is decreased by _________________
Aplastic anemia, aplastic crisis, ineffective erythropoiesis, and medications
____ ________ should be confined with wrights stain because they can falsely increase retic count
RBC inclusions
Sickle cell screening is for ______ __ detection
Hgb S
Reagent is prepared by mixing ________ _________ with __________ __________
phosphate buffer with sodium hydrosulfide
With Sickle cell screening, disease with high _______ _________ may produce a false-positive
plasma protein