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Hematology-Exam 1 > Hemolytic Anemias > Flashcards

Hemolytic Anemias Flashcards

1
Q

What is the disorder characterized by decreased red cell lifespan?

A

hemolytic anemia

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2
Q

What is the lifespan of normal RBCs?

A

120 days (4 months)

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3
Q

Jaundice, pigmented gallstones, ankle ulcers, splenomegaly, and aplastic crises associated with Parvovirus B19, increased folate requirement are clinical presentations of

A

Hemolysis

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4
Q

Yellowing of sclera is known as

A

scleral icterus

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5
Q

more generalized yellow discoloration of tissues is known as

A

jaundice

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6
Q

jaundice is caused by ____ which is a byproduct of hemoglobin breakdown

A

bilirubin

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7
Q

What can be found on peripheral blood smear of RBCs post splenectomy as an indication that the spleen has grown back?

A

Howell-jolly bodies (small round blue DNA remnants)

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8
Q

What organism can cause aplastic crisis in patients with hemolytic anemias by halting erythropoiesis for a week?

A

Parvovirus B19 (non-encapsulated DNA virus)

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9
Q

3 classifications of hemolytic anemia

A

sites of RBC destruction
acquired v. congenital
mechanism of red cell damage

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10
Q

Differentiate between extravascular v. intravascular hemolysis

A
  • Extravascular has macrophages in spleen, liver, and marrow remove damaged RBCs.
  • Intravascular has RBCs rupture “within” vasculature, releasing free Hb into circulation
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11
Q

What are the evidences for increased RBC production?

A
  1. Elevated reticulocyte count (in blood)
  2. Erythroid Hyperplasia (in bone marrow)
  3. Skeletal deformities (frontal bossing in bones)
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12
Q

What are outcomes/consequences of hemolysis?

A
  1. Elevated LDH (lactate dehydrogenase; released after any cell lysis)
  2. Elevated Unconjugated Bilirubin
  3. Reduced serum haptoglobin
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13
Q

What is glycosylated hemoglobin that is used as a measure of glucose control?

A

Hb A1C

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14
Q

In patients with hemolysis, Hb A1C can be

A

falsely low (because hemoglobin doesn’t last long enough to get glycosylated)

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15
Q

Most common defect leading to anemia

A

hereditary spherocytosis (autosomal dominant)

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16
Q

What pinches off in hereditary spherocytosis? What is the defect causing this?

A
  • Lipid microvesicles (in the spleen) pinch off

- Defect in ankyrin (RBC membrane cytoskeleton) leads to loss of SA and more spherocytic shape

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17
Q

How is hereditary spherocytosis diagnosed?

A
  • Decreased Eosin-5’ Maleimide Flow cytometry staining

- Increased Osmotic fragility

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18
Q

2 treatment options for hereditary spherocytosis

A
  1. folate supplementation

2. splenectomy

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19
Q

What test can be beneficial to spherocytosis?

A

MCHC (can be elevated)

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20
Q

What is rate-limiting enzyme in pentose-phosphate shunt?

A

G6PD

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21
Q

A defect in G6PD results in low levels of _____ and reduced _____ which protect hemoglobin from oxidative damage.

A

NADPH; glutathione

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22
Q

In the absence of G6PD, hemoglobin is converted to methemoglobin and eventually precipitate as

A

Heinz Bodies

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23
Q

Heinz Bodies are pinched off by ____ and become _____

A

spleen; bite cell (or blister cell)

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24
Q

Genetic transmission of G6PD deficiency

A

X-linked

25
Q

anti-malarials, sulfa drugs, dapsone, Vitamin K, fava beans, Naphtha (mothballs) are agents to avoid in

A

G6PD deficiency

26
Q

In patients with G6PD deficiency, hemolysis is trigger by

A

drugs or infections

27
Q

How can AA patients with G6PD deficiency may have normal G6PD levels?

A

Mature cells have been lysed and only younger cells (reticulocytes) with normal G6PD levels are left.

28
Q

What are lab findings of G6PD deficiency?

A
  1. Bite and Blister cells

2. Low G6PD Levels

29
Q

What are examples of acquired hemolytic anemias?

A

AIHA (warm & cold); PNH

30
Q

hallmark of autoimmune hemolytic anemia

A

Positive Coomb’s Test

31
Q

Describe characteristics of warm antibodies.

A

React best at 37C (body temp.), IgGs are the antibodies, do not agglutinate RBCs

32
Q

Describe characteristics of cold antibodies.

A

React best <32C (extremities), IgMs are the antibodies, cause RBC agglutination

33
Q

What does the Direct Coomb’s test look for?

A

Tests for IgG or C3 that are bound DIRECTLY on the RBCs

34
Q

What does Coomb’s reagent consist of?

A

antibodies to human IgG and C3

35
Q

True or False: Agglutination in the “test tube” is NOT the same as cold antibodies agglutination disease

A

True

36
Q

Smear finding in warm-antibody hemolytic anemias

A

spherocytes

37
Q

AIHA can be induced by

A

drugs

38
Q

AIHA is what type of hypersensitivity?

A

Type II

39
Q

Splenomegaly, jaundice, and anemia are clinical presentations of

A

warm-antibody AIHA

40
Q

Anemia of warm-antibody AIHA can be VERY symptomatic depending on

A

rate of fall in hemoglobin

41
Q

Positive coomb’s test, spherocytes , elevated MCHC are lab findings of

A

warm-antibody AIHA

42
Q

What is the mainstay of therapy for warm -antibody AIHA?

A

immunosuppressant drugs

  • 1st line: corticosteroids
  • 2nd: Rituximab
  • 3rd: splenectomy
43
Q

What is the main mechanism of warm-antibody AIHA?

A

IgG or/and C3 bind to RBCs and they are removed by macrophages (have Fc and C3b receptors)

44
Q

What is the main mechanism of cold-agglutinin AIHA?

A

IgM bind to RBCs in the cooler extremities and then fix complement. Complement-coated RBCs are lysed within the vessel (intravascular hemolysis) or by macrophages (extravascular hemolysis).

45
Q

How can IgM lead to RBC agglutination?

A

because IgM is pentameric

46
Q

After forming cold agglutinins, what can happen if they cannot pass through microvasculature?

A

Distal Occlusion (cyanosis and ischemia in extremities)

47
Q

What can be seen on smear of cold agglutinin disease?

A

RBC agglutination

48
Q

What infections and disease are associated with cold agglutinin disease?

A

mycoplasma and mononucleosis

lymphoproliferative disease

49
Q

Positive coomb’s test for C3 but not IgG, RBC agglutination are the lab findings of

A

cold-antibodies (cold-agglutinin) AIHA

50
Q

Treatment of cold agglutinin disease

A

keep patient warm (immunosuppressants are INEFFECTIVE)

51
Q

Hallmark of microangiopathic hemolytic anemia

A

presence of schistocytes on peripheral smear

52
Q

TTP/HUS and DIC are the causes of

A

microangiopathic hemolytic anemia

53
Q

What is an acquired clonal disorder of hematopoietic stem cells?

A

Paroxysmal Nocturnal Hemoglobinuria (PNH)

54
Q

What is the cause of PNH?

A

A defect in PIG-A gene that makes GPI proteins that anchor other protein on the cell surface.

55
Q

What are the two GPI proteins that can lead to PNH due to PIG-A defect making them?

A

CD55 (DAF)

CD59

  • their normal function is to inhibit complement; defect leads to complement mediated RBC destruction (hemolysis)
56
Q

PNH-derived hemolysis usually occurs

A

at night when the pH falls

57
Q

The smooth msucle relaxation symptoms (esophageal spasm, erectile dysfunction, pulmonary hypertension) can occur from intravascular hemolysis of PNH due to

A

low nitric oxide levels

58
Q

What is the treatment for PNH?

A

Eculizumab

*targets C5 and blocks terminal complement activation

59
Q

What is the risk of using Eculizumab?

A

Increased susceptibility to Neisseria infection.

Hematology-Exam 1 (12 decks)

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