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Anemia Pt. 2 Electric Boogaloo > It's Hemo-Lit-ic Fam > Flashcards

It's Hemo-Lit-ic Fam Flashcards

1
Q

Which of these are not indicators of hemolysis?

a. Increased bilirubin
b. Decreased hemosiderin in macrophages
c. Decreased reticulocytes
d. Decreased haptoglobin
e. Increased isoenzymes 1 & 2

A

B & C are both false, they both show increased levels during hemolysis

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2
Q

Why can RBC indices sometimes not reflect the presence of anemia during acute blood loss?

A
  • Both plasma and rbcs are lost simultaneously, so RBC indices are decreased relative to each other.
  • Body has not had time to fully compensate with fluid retention
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3
Q

How can Hydremia affect RBC indices?

A

Increase in plasma volume, which can lead to decreased H&H and “appearing” as anemic on paper

Hydro- (water related) & -emia (involving blood)

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4
Q

What can cause Hydremia?

A
  • Hyperproteinemia (which can also cause a false Pos SickleDex tube test)
  • Pregnancy
  • Massive IV or FFP infusion
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5
Q

What are the 4 main groups of RBC membrane defects?

A

HS
HE (includes Pyropoik- and SE Asian Ovalocytosis)
Hereditary Stomatocytosis
Hereditary Xerocytosis

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6
Q

Which 2 groups of membrane defects are caused by horizontal spectrin-ankyrin defects?

A

HE and Pyropoikilocytosis

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7
Q

Which membrane defect group is caused by vertical spectrin-ankyrin defects?

A

HS

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8
Q

Classical Presentations of HS include:

A
  • Jaundice
  • Splenomegaly
  • Anemia (mild to severe)
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9
Q

What result do you expect to see in an osmotic fragility test with HS?

A

Increased osmotic fragility

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10
Q

True or False
EDTA tubes can be used to collect samples for an osmotic fragility test?

A

False
Heparin Tubes are used

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11
Q

What is the expected result of an osmotic fragility of a normal RBC?

A

Normal RBCs will begin to lyse at .45% saline and fully lyse at .35% saline.

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12
Q

Which types of cells are associated with a decreased osmotic fragility?

A

Target Cells

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13
Q

What other lab findings (including RBC indices) are seen with HS?

A
  • DAT negative
  • Folic Acid Decreased
  • MCV Decreased to Norm
  • MCHC Increased, often >36
  • Retics Increased (polychromasia)
  • RDW Increased
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14
Q

What’s the range of severity for HE?

A

Silent carrier to severe

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15
Q

Classical Presentations of HS include:

A
  • Jaundice
  • Splenomegaly
  • Anemia (mild to severe)
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16
Q

What other lab findings (including RBC indices) are seen with HE?

A
  • Elliptocytes
  • MCV Normal to Increased
  • MCHC Normal
  • Osmotic Fragility Normal
  • Some may have mildly heat sensitive RBCs
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17
Q

What’s the treatment for all HE cases?

A

Splenectomy

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18
Q

What hallmark is seen in Southeast Asian Ovalocytosis?

A

Spoon shaped ovalocytes with a transverse bar across central pallor space (think double stomatocyte)

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19
Q

What two defect types are associated with Hereditary Pyropoikillocytosis?

A
  • Alpha or Beta horizontal spectrin mutation
  • Decreased synthesis of alpha spectrin
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20
Q

True or False
Splenectomy fully resolves hemolysis in Pyropoik

A

False
Some hemolysis remains and some cells may fragment at body temp

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21
Q

What population is most affected by HPP and what symptoms are generally seen?

A
  • Black populations are mostly affected
  • Symptoms seen include Facial bone deformities, Gallbladder Disease, and stunted growth
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22
Q

What Lab Findings are seen when observing HPP sample results (CBC/Diff)?

A
  • MCV Very Decreased
  • MCHC Usually Increased
  • Bizarre Micro Poik (RBC Budding & Fragments, other bizarre shaped rbcs)
  • Elliptocytes
  • Spherocytes
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23
Q

What deficiency do most patients with Hereditary Stomatocytosis show and what is its theorized function?

A

Deficiency of Stomatin
Theorized to regulate an unidentified ion channel

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24
Q

What causes Hereditary Stomatocytosis?

A

Failure of Na/K pumps leading to increased H2O influx to cells and swelling.

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25
Q

What’s the treatment for Hereditary Stomatocytosis?

A

Splenectomy

26
Q

What’s the range of hemolysis for Stomatocytosis?

A

Mild to Severe

27
Q

What Lab Findings are seen with Hereditary Stomatocytosis (CBC/Diff & others)?

A
  • MCV Increased
  • MCHC Decreased bc of H2O dilution
  • Abundant stomatocytes
  • Osmotic Fragility Increased
  • Decreased 2,3-BPG (leads to increased O2 affinity in Hgb and decreased O2 delivery)
28
Q

What are some of the causes of Acquired Stomatocytosis?

A
  • Acute Alcoholism
  • Drug therapy
  • Liver disease
  • Cardiovascular disease
  • Occasionally in marathon runners after races
29
Q

What defect causes Xerocytosis and what happens to the RBCs?

A

Unknown defect causes K loss bc of RBC permeability defect
Cells dehydrate and become raisins (not really lol)

30
Q

True or False
Splenectomy can partially resolve Xeroctosis hemolysis

A

False
It doesn’t correct the hemolysis bc they aren’t sequestered in the spleen

31
Q

What are the Lab Findings for Xerocytosis?

A
  • MCV Increased due to macrocytes
  • MCHC Increased
  • Stomatocytes
  • Target Cells
  • “Puddle Cells” (think the “Signet Rings of RBCs”)
  • Decreased 2,3-BPG
  • Decreased Osmotic Fragility
32
Q

Which type of Hereditary Group abnormality is caused by abnormal membrane lipids?

A

Acanthocytosis

33
Q

In what population of cells is acanthocytosis shape defect not seen in?

A

Shape defect is not present in young RBCs

34
Q

What is the cause associated with hereditary acanthocytosis?

A

Abetalipoproteinemia

35
Q

What are the non-hereditary causes (or the ones we didn’t focus on) for Acanthocytosis?

A
  • Liver disease
  • Malnutrition
  • Certain Blood groups (McCloud, related to Kel and Lutheran)
  • Vit E deficiency
36
Q

What’s the general appearance of RBCs in Liver Disease Associated Anemia?

A

Target Cells, Round Macrocytes, Acanthocytes

37
Q

What’s the most common enzyme deficiency associated with the E-M Pathway?

A

P-K Deficiency

38
Q

What inclusions are seen with oxidative denaturation of hemoglobin?

A

Heinz Bodies

39
Q

How is the rate of reticulocytosis associated with hemolysis in G-6-PD deficiencies?

A

Inversely proportional, older cells with less enzyme are hemolyzed and the younger cells survive and reign supreme.

40
Q

What’s the most common hereditary enzyme deficiency that causes anemia?

A

G-6-PD Deficiency (x-linked)

41
Q

What is Favism?

A

Fava beans causing oxidative damage and incurring a hemolytic crisis in G-6-PD deficient pts

42
Q

What CBC/Diff results do you expect to see in G-6-PD deficiency?

A
  • Reticulocytes Greatly Increased
  • Heinz Bodies on a Supravital Stain
  • Generally normo/normo
  • Morphology changes based on amount of hemolysis
43
Q

What does a positive G-6-PD deficiency result look like under UV testing?

A

Positive result for deficiency will show NO fluorescence

44
Q

In a PK Deficiency, what are the expected Lab Findings?

A
  • Anemia (fully compensated to severe range)
  • Increased 2,3-BPG (increased O2 delivery)
  • Increased Reticulocytes
  • Generally normal rbc morphology
45
Q

How does splenectomy affect PK-deficient hemolysis?

A

It doesn’t correct hemolysis, but does usually increase Hgb by 1-3 grams

46
Q

What does a positive PK deficiency result look like under UV testing?

A

Positive PK deficiency result will show fluorescence under UV light

47
Q

What causes hemolysis in PNH?

A

Complement fixation to RBCs due to a membrane defect of decreased GPI anchor protein (especially in low pH and reduced ionic strength environments, ie nighttime)

48
Q

What Lab Findings are seen with PNH?

A
  • Possible development of IDA or Aplastic Anemia (if the latter wasn’t there already)
  • Thrombosis
  • Thrombocytopenia
  • Neutropenia
  • Dual population of RBCs (malignant and normal)
  • Hemoglobinemia (with possible hemoglobinurea)
  • Hemosiderinurea
49
Q

How can PNH be tested for?

A

Flow cytometry for surface expression of GPI-anchoring proteins on RBCs and Granulocytes

50
Q

What’s the difference between DIC and MAHA?

A

MAHA is a chronic condition, while DIC is usually considered to be acute.

51
Q

What two disorders are associated with MAHA?

A

TTP and HUS

52
Q

What RBC characteristics are seen with pts post-splenectomy?

A

HJ Bodies, Target Cells, Siderocytes

53
Q

Name the pentad of findings associated with TTP

A
  • Hemolytic anemia with schistocytes
  • Thrombocytopenia (hemorrhage)
  • Fluctuating Neurological Signs
  • Fever
  • Progressive Renal Disease
54
Q

What is the surmised cause of small platelet agrgregates and subsequent occlusions in organ capillaries with TTP?

A

Unusually large VonWillebrand Factor present on epithelial cells that would normally be cleaved by circulating ADAMSTS13

55
Q

Acquired TTP is defined by an:

A

Auto-antibody to ADAMSTS13
-or-
a rare inherited variant of TTP caused by ADAMSTS13 deficiency

56
Q

What is the Triad of findings present in HUS?

A
  • MAHA
  • Thrombocytopenia
  • Acute Renal Failure
57
Q

How is TTP differentiated from HUS?

A

HUS has an acute renal failure aspect while TTP is a chronic renal failure
HUS lacks neurological symptoms

58
Q

What is the general principle of HUS?

A

Follows enteric infections
Toxins target renal capillary endothelium
May set off DIC/local fibrinolysis doesn’t occur
May cause plt activation

59
Q

How do an aortic valve replacement and March Hemoglobinuria cause hemolytic anemia?

A
  • Non-flexible implant leads to mechanical damage to RBCs
  • Continued stress to RBCs and vasculature due to nonstop marching (I assume?) - no schistocytes usually seen
60
Q

What microorganisms can cause hemolytic anemia?

A
  • Malaria
  • Babesia
  • Bartonella
  • Clostridia
  • Strep
61
Q
A

Anemia Pt. 2 Electric Boogaloo (3 decks)

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