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IBI Test 2 Leesy > B3.035 - Hemolytic Anemias Prework 1 > Flashcards

B3.035 - Hemolytic Anemias Prework 1 Flashcards

1
Q

What does intrinsic hemolytic anemia mean

A

defect in RBC itself

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2
Q

What does extrinsic hemolytic anemia mean

A

defect/problem outside RBC but affects RBC

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3
Q

What does intravascular hemolytic anemia mean

A

RBC breakdown with massive release of free Hgb in circulation

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4
Q

What does extravascular hemolytic anemia mean

A

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

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5
Q

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

A

Decreased deformability of cell and yes

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6
Q

what causes reticulocytosis

A

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

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7
Q

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

A

increased

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8
Q

In EH what happens to LDH

A

increased

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9
Q

In EH unconjugated bilirubin is

A

increased

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10
Q

In EH haptoglobin is

A

Decreased or normal

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11
Q

In EH serum free hemoglobin is

A

Absent

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12
Q

In EH urine hemoglobin is

A

Absent

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13
Q

In IH reticulocyte count, EPO, polychromasia is

A

increased

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14
Q

In IH LDH is

A

Increased

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15
Q

In IH unconjugated Hgb is

A

Increased

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16
Q

In IH haptogobin is

A

decreased

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17
Q

In IH serum free Hgb is

A

present

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18
Q

In IH urine Hgb is

A

present

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19
Q

What diseases have intrinsic etiology

A

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

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20
Q

What diseases have extrinsic etiology

A

Autoimmune hemolytic anemia
Traumatic hemolytic anemia
infections like malaria

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21
Q

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

A

Reticulocytosis
anemia
blood smear morphology
Intravascular vs extravascular

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22
Q

Most hemolytic anemias are what -cytic

A

normocytic

23
Q

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

A

Thalassemias is microcytic and hypochromic

24
Q

what disease is characterized by sphistocytes

A

microangiopathic hemolytiv anemia

25
Q

what disease is characterized by sperocytes

A

hereditary spherocytosis, warm autoimmune hemolytic anemia

26
Q

what disease is characterized by RBC agglutination

A

cold autoimmune hemolytic anemia

27
Q

what are spherocytes

A

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

28
Q

what is hemoglobinopathy (eg. sickle cell)

A

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

29
Q

what is thalassemia

A

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

30
Q

what are important proteins of the cytoskeleton of RBCs

A

Ankyrin, spectrin, band 3, band 4.2

31
Q

what is spherocytosis

A

membrane defect in cytoskeletal protein

Extravascular, intrinsic, inherited

32
Q

what is an RBC enzyme defect

A

G6P dehydrogenase deficiency

33
Q

what is G6P dehydrogenase deficiency

A

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

34
Q

what is the inhertiance pattern of hereditary spherocytosis

A

Ankyrin is the culprit usually and AD

35
Q

what is G6PD used for

A

reducing GSH

36
Q

what is PNH

A

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

37
Q

what does PIGA do

A

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

38
Q

what proteins help anchor RBCs to PIGA

A

CD55, CD59

39
Q

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

A

Intravascular intrinsic aquired

40
Q

patients with PNH are at risk of what

A

blood organ cancers

41
Q

what are the autoimmune hemolytic anemias

A

Warm autoimmune hemolytic anemia

Cold autoimmune hemolytic anemia

42
Q

what is warm AHA

A

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

43
Q

what is cold AHA

A

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

44
Q

how do you detect antibodies coating RBCs

A

coombs test

45
Q

how do you treat cold AHA?

A

keep them warm!

46
Q

what is direct coombs test

A

detects Abs coating RBCs

47
Q

What is indirect coombs test

A

detects Ab in plasma that can react with RBCs

48
Q

what is DAT

A

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

49
Q

what are allimunization and transfusion reaction for

A

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

50
Q

alloantibodies to other RBC antigens cause what

A

IgG, extravascular hemolysis over weeks

51
Q

What is traumatic hemolytic anemia caused by

A

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

52
Q

describe trauma in microcirculation

A

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

53
Q

what are found in trauma in microcirculation

A

schistocytes thrombocytopenia cariable abnormalities of coagulation tests
intravascular extrinsic aquired

IBI Test 2 Leesy (13 decks)

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