B3.035 - Hemolytic Anemias Prework 1

Question 1

What does intrinsic hemolytic anemia mean

Answer 1

defect in RBC itself

Question 2

What does extrinsic hemolytic anemia mean

Answer 2

defect/problem outside RBC but affects RBC

Question 3

What does intravascular hemolytic anemia mean

Answer 3

RBC breakdown with massive release of free Hgb in circulation

Question 4

What does extravascular hemolytic anemia mean

Answer 4

RBC breakdown in reticuloendothelial system cells (macrophages in spleen and liver) with capture of Hgb

Question 5

What is extravascular hemolytic anemia usually caused by and does any Hgb get into circulation?

Answer 5

Decreased deformability of cell and yes

Question 6

what causes reticulocytosis

Answer 6

anemia decreases RBC mass which stimulates kidney to release erythropoetin which stimulates bone marrow to increase amount of reticulocytes

Question 7

In EH what are the reticulocyte count, EPO, polychromasia labs going to show

Answer 7

increased

Question 8

In EH what happens to LDH

Answer 8

increased

Question 9

In EH unconjugated bilirubin is

Answer 9

increased

Question 10

In EH haptoglobin is

Answer 10

Decreased or normal

Question 11

In EH serum free hemoglobin is

Answer 11

Absent

Question 12

In EH urine hemoglobin is

Answer 12

Absent

Question 13

In IH reticulocyte count, EPO, polychromasia is

Answer 13

increased

Question 14

In IH LDH is

Answer 14

Increased

Question 15

In IH unconjugated Hgb is

Answer 15

Increased

Question 16

In IH haptogobin is

Answer 16

decreased

Question 17

In IH serum free Hgb is

Answer 17

present

Question 18

In IH urine Hgb is

Answer 18

present

Question 19

What diseases have intrinsic etiology

Answer 19

Hemoglobinopathy - sickle cell
Thalassemia (microcytic) - alpha and beta
Membrane abnormality - heredetrary spherocytosis
RBC enzyme defects - G6P dehydrogenase deficiency
Paroxysmal nocturnal hemoglobinuria

Question 20

What diseases have extrinsic etiology

Answer 20

Autoimmune hemolytic anemia
Traumatic hemolytic anemia
infections like malaria

Question 21

when looking for hemolytic anemia what lap features would you look for

Answer 21

Reticulocytosis
anemia
blood smear morphology
Intravascular vs extravascular

Question 22

Most hemolytic anemias are what -cytic

Answer 22

normocytic

Question 23

what is the exception to the rule that hemolytic anemias are normocytic and what type is it

Answer 23

Thalassemias is microcytic and hypochromic

Question 24

what disease is characterized by sphistocytes

Answer 24

microangiopathic hemolytiv anemia

Question 25

what disease is characterized by sperocytes

Answer 25

hereditary spherocytosis, warm autoimmune hemolytic anemia

Question 26

what disease is characterized by RBC agglutination

Answer 26

cold autoimmune hemolytic anemia

Question 27

what are spherocytes

Answer 27

when pieces of RBC membrane get taken up by splenic macrophages, RBC goes from biconcave shape to sphere

Question 28

what is hemoglobinopathy (eg. sickle cell)

Answer 28

structurally abnormal hemoglobin due to mutation in alpha or beta globin gene Extravascular, intrinsic, inherited

Question 29

what is thalassemia

Answer 29

Decreased production of alpha or beta chains due to mutations Extravascular, intrinsic, inherited

Question 30

what are important proteins of the cytoskeleton of RBCs

Answer 30

Ankyrin, spectrin, band 3, band 4.2

Question 31

what is spherocytosis

Answer 31

membrane defect in cytoskeletal protein | Extravascular, intrinsic, inherited

Question 32

what is an RBC enzyme defect

Answer 32

G6P dehydrogenase deficiency

Question 33

what is G6P dehydrogenase deficiency

Answer 33

decreased level of critical enzyme, increased sensitivity to oxidative stress Intravascular and extravascular intrinsic inherited XR

Question 34

what is the inhertiance pattern of hereditary spherocytosis

Answer 34

Ankyrin is the culprit usually and AD

Question 35

what is G6PD used for

Answer 35

reducing GSH

Question 36

what is PNH

Answer 36

clonal disorder with mutation in phosphatidylinositol glycan complementation group A (PIGA) gene

Question 37

what does PIGA do

Answer 37

its an anchor protein that works to prevent complement mediated lysis of RBCs

Question 38

what proteins help anchor RBCs to PIGA

Answer 38

CD55, CD59

Question 39

describe PNH (intra/extra, intrinsic/extrinsic, aquired/hereditary)

Answer 39

Intravascular intrinsic aquired

Question 40

patients with PNH are at risk of what

Answer 40

blood organ cancers

Question 41

what are the autoimmune hemolytic anemias

Answer 41

Warm autoimmune hemolytic anemia | Cold autoimmune hemolytic anemia

Question 42

what is warm AHA

Answer 42

IgG antibodies active at 37 degrees recognized by splenic macrophages (Fc receptors) with membarne loss (Spherocytes) extravascular extrinsic aquired

Question 43

what is cold AHA

Answer 43

IgM antibodies active below 37 degrees, large pentameric molecule can bind to more than 1 RBC (agglutination)and activate complement with complement mediated RBC lysis intravascular and extravascular extrinsic aquired

Question 44

how do you detect antibodies coating RBCs

Answer 44

coombs test

Question 45

how do you treat cold AHA?

Answer 45

keep them warm!

Question 46

what is direct coombs test

Answer 46

detects Abs coating RBCs

Question 47

What is indirect coombs test

Answer 47

detects Ab in plasma that can react with RBCs

Question 48

what is DAT

Answer 48

identifies IgG or IgM Abs coating RBCs, can differentiate etiology of spherocytes

Question 49

what are allimunization and transfusion reaction for

Answer 49

ABO antibodies, is what happens when you transfuse a blood type other than your own would cause massive intravascular hemolysis

Question 50

alloantibodies to other RBC antigens cause what

Answer 50

IgG, extravascular hemolysis over weeks

Question 51

What is traumatic hemolytic anemia caused by

Answer 51

trauma in large vessels/heart (macroangiopathic) intravascular extrinsic aquired trauma in microcirculation

Question 52

describe trauma in microcirculation

Answer 52

trauma due to widespread clots in microcirculation Platelet thrombi - TTP, HUS Platelet fibrin thrombi - DIC

Question 53

what are found in trauma in microcirculation

Answer 53

schistocytes thrombocytopenia cariable abnormalities of coagulation tests intravascular extrinsic aquired