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Hematology Exam 1 > Hemolytic Anemias > Flashcards

Hemolytic Anemias Flashcards

1
Q

IgG-coated RBCs interact with __ receptors on macrophages and that causes _____ hemolysis and formation of _____

A

Fc; extravascular; spherocytes

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2
Q

Bite cells are derived from ____ _____

A

Heinz bodies

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3
Q

In HA, the bone marrow expands due to increased erythropoiesis. What bones are likely to be expanded?

A

Skull, ribs, long bones

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4
Q

____ is released after lysis of any cell

A

LDH

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5
Q

What should be given to patients prior to splenectomy?

A

Vaccination against encapsulated organisms

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6
Q

What cells will you see in a blood smear of someone with hereditary spherocytosis?

A

Spherocytes

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7
Q

Is a splenectomy or corticosteroids effective in treating in cold agglutinin disease?

A

NO

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8
Q

In African variant of G6PD deficiency, will the old or new RBCs be more unstable?

A

The old

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9
Q

Complement-coated red cells can undergo ________ and ______ hemolysis

A

Intra and extravascular

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10
Q

Direct Coomb’s= DAT and this tests for ___ or ___ which are bound directly to RBC

A

IgG; C3

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11
Q

Heinz bodies are indicative of what deficiency?

A

G6PD

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12
Q

What is the rate limiting enzyme in the Pentose-phosphate shunt?

A

G6PD

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13
Q

Methemoglobin is capable/incapable of carrying O2 to tissues

A

incapable

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14
Q

Patients with hereditary spherocytosis get increasingly anemic and have abdominal pain during ____ infections

A

Mild

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15
Q

If you do a Coomb’s test and there is agglutination in the tube then the test is _____ for IgG or ___

A

Positive; C3

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16
Q

Can get infection with mycoplasma or mononucleosis and develop ________

A

Cold agglutinin disease that will resolve when the infection is cleared

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17
Q

Why can digital ischemia occur in a patient with in cold agglutinin disease?

A

IgM causes agglutination of the red cells and they cannot pass through the microvasculature and that causes ischemia and cyanosis

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18
Q

In AIHA, what are some clinical manifestations that will be seen?

A 
Splenomegaly
High reticulocytes
High LDH
High bilirubin
Low haptoglobin
Positive Coomb's test
Spherocytes
Can be elevated MCHC
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19
Q

In someone with G6PD deficiency, what are some oxidant agents to avoid?

A
  • Quinine
  • Sulfa drugs
  • Dapsone
  • Vitamin K
  • Fava Beans
  • Naphtha (moth balls)
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20
Q

What percentage of African American men have G6PD deficiency?

A

10-14%

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21
Q

In someone with the African variant of G6PD deficiency, the G6PD levels after a hemolytic will be normal/abnormal

A

Normal because the old cells that have lysed were deficient and the new cells are not

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22
Q

AIHA can be provoked by certain infections and induced by ___

A

drugs

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23
Q

Yellowing of sclera in the eyes is referred to as:

A

scleral icterus

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24
Q

Unconjugated/conjugated bilirubin is elevated in HA

A

Unconjugated

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25
Q

Erythroid hyperplasia is characteristic of what kind of anemia?

A

Hemolytic; myeloid:erythroid ratio is usually 3:1 and in hyperplasia, the ratio is 1:10

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26
Q

Low G6PD results in low levels of glutathione and ____ which are necessary to protect Hb from oxidative damage

A

NADPH

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27
Q

An African American child that has come into the ER and has breath that smells of moth balls may be undergoing ____

A

hemolysis due to G6PD deficiency

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28
Q

A child with a deformed skull and high reticulocyte counts may be suffering from what consequence of B-thalassemia major?

A

Frontal bossing

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29
Q

When the reticulocyte count is close to 0, this is referred to as an/a _____ crisis

A

aplastic

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30
Q

In hereditary spherocytosis, the MCHC can be _______

A

Elevated

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31
Q

G6PD deficiency leads to hemoglobin conversion to ______ and the production of ____ bodies and the spleen pinches this off and overlying membrane and leaves a ____ cell

A

methemoglobin; Heinz; bite

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32
Q

Positive DAT for C3 and negative for IgG=

A

Cold agglutinin disease

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33
Q

Dapsone is used for treatment of _____ and prophylaxis for ___

A

Leprosy, PJP

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34
Q

A myeloid:erythroid ratio of 1:10 would indicate what?

A

Erythroid hyperplasia

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35
Q

A patient who has chronic hemolytic anemia presents with an acute drop in their baseline hemoglobin value. What is the likely diagnosis and what would you test for?

A

Aplastic crisis; check retic count and send in PCR for parvovirus

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36
Q

Fingertips, toes, and tip of nose are places where ___ binds red cells in cold agglutinin disease and then fixes ________

A

IgM; complement

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37
Q

Normal red cell lifespan

A

120 days

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38
Q

Increased osmotic fragility is a test for ______ _______

A

hereditary spherocytosis

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39
Q

C3-coated red cells interact with C3 receptors on ______ and this causes _______ hemolysis

A

Extravascular

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40
Q

Lipid microvesicles (blebs) form on the surface of the RBCs in hereditary spherocytosis and they are pinched off in the _____ by ______ and this is referred to as ________ hemolysis

A

spleen; macrophages; extravascular

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41
Q

Lactate dehydrogenase (LDH) is found in some/all cells

42
Q

An adult with parvovirus infection may present with which 2 key clinical manifestations?

A

Fever and arthritis of the hands

43
Q

The complement cascade may cause lysis of the cells and that leads to ______ hemolysis

A

Intravascular

44
Q

Why is infection with parvovirus B19 so dangerous in patients with hemolytic anemia?

A
  • parvovirus B19 suppresses bone marrow erythropoietic activity
  • patients with HA have RBCs that only last about 10 days and the virus may persist that long and suppress production of new RBCs so that leads to an aplastic crisis
45
Q

End result of Hb breakdown is ______.

46
Q

Blister cells are seen in patents with what condition?

A

G6PD deficiency

47
Q

Hereditary spherocytosis is caused by defects in the proteins of the membrane skeleton, usually _____

48
Q

Macrophages remove damaged or red cells coated with _______

A

Antibodies

49
Q

Haptoglobin is made in the _____ and has a half life of ____ days

50
Q

_______ is a byproduct of heme breakdown and the elevated portion is _______

A

Bilirubin; unconjugated

51
Q

Where is G6PD deficiency seen and why?

A

In Africa and the Mediterranean; some malarial resistance

52
Q

If there are reduced levels of haptoglobin, what type of hemolysis is occurring?

A

Intravascular

53
Q

In cold agglutinin disease, when IgM and complement fixed red cells travel to warm parts of the body, ____ falls off

54
Q

White/red cells turn over more quickly are are more predominant in the marrow

A

White cells (myeloid)

55
Q

Cold agglutinin disease may be associated with

A

lymphoproliferative disease

56
Q

This type of anemia is characterized by decreased red cell lifespan

57
Q

______ _____ is the most common congenital defect leading to anemia

A

Hereditary spherocytosis

58
Q

An increase in this biochemical breakdown product increases in HA patients

59
Q

Blister and ____ cells are present on peripheral blood smear of someone with G6PD deficiency

60
Q

What is the underlying problem of the RBCs in HA?

A

Destruction of RBCs

61
Q

A Direct Coomb’s test will be positive for C3 in patients with this disease

A

Cold agglutinin disease

62
Q

Warm antibodies do not cause ______

A

agglutination

63
Q

Will you likely see frontal bossing in mild HA?

A

No, mainly seen in severe HA (i.e. B-thalassemia)

64
Q

How could you treat someone with hereditary spherocytosis?

A

Folate supplementation

Splenectomy

65
Q

A child with parvovirus infection may present with which 2 key clinical manifestations?

A

Fever and rash; Fifth disease

66
Q

What are some key clinical manifestations of someone with hemolytic anemia?

A
  • Jaundice
  • Tea-colored urine
  • Pigmented gallstones
  • Ankle ulcers
  • Splenomegaly (generally)
67
Q

Bilirubin normally passes through the _____ and is conjugated to glucoronide and is stored in the gallbladder before being released in the stool

68
Q

The ___ levels will increase in HA, but it is not very specific

69
Q

If someone has HIV and want to start them on sulfa drugs or dapsone, what levels should you check first?

70
Q

Anaerobic glycolysis pathway in RBCs

A

Embden-Myerhof pathway

71
Q

Quinine is used for

A

Anti-malaria treatment

72
Q

In a reduced osmotic concentration a spherocyte will ___ while a normal red cell will not

73
Q

What is the inheritance pattern of hereditary spherocytosis?

A

Autosomal dominant

74
Q

What are effective treatments for cold agglutinin disease?

A

Keep patient warm; Rituximab

75
Q

What is the treatment for warm AIHA?

A

Immunosuppression mainly or transfusion if indicated and folate repletion

76
Q

G6PD reduces/oxidizes hemoglobin

77
Q

If someone with hereditary spherocytosis undergoes a splenectomy, the hemolysis will decrease, but what will stay the same?

A

The spherocytes are still there because it is a genetic defect in the membrane protein

78
Q

In HA, the RDW should be increased/decreased

79
Q

Which variant of G6PD deficiency is more severe: African or Mediterranean?

A

Mediterranean

80
Q

_______ are used as the first line of treatment of AIHA and if it does not work, then may perform a splenectomy and/ or use ______

A

Corticosteroids (Prednisone); Rituximab

81
Q

A positive Coomb’s test=

A

Autoimmune Hemolytic Anemia (AIHA)

82
Q

When haptoglobin binds to free hemoglobin, the half life increases/ decreases

A

Decreases; about 20 min

83
Q

Hereditary spherocytosis affects mainly what ancestry?

A

1/5000 of European descent

84
Q

AIHA can manifest as a primary disorder with no underlying problem or it can be complication of other diseases such as ____ or CLL or Non-Hodgkin’s lymphoma

85
Q

Pentose-phosphate shunt starts with which molecule?

86
Q

In Hereditary spherocytosis, there is a decrease in the ___, due to loss of membrane

87
Q

G6PD deficiency is what inheritance pattern?

88
Q

In intravascular hemolysis, can see reduced ______, hemoglobinemia, ________, and hemosiderinuria

A

haptoglobin; hemoglobinuria

89
Q

During intravascular hemolysis, red cells rupture within the ________ and that releases free _____ into circulation

A

vasculature; hemoglobin

90
Q

Aerobic pathway used in RBCs

A

Pentose-phosphate shunt

91
Q

MAHA characteristic cells found on smear are

A

Schistocytes

92
Q

Cold antibodies cause _____

A

agglutination

93
Q

Which Ig works best at <32C?

94
Q

An increased number of reticulocytes produces a blood smear with a lot of ______

A

polychromasia

95
Q

Reduced serum _________ is a sensitive marker for intravascular hemolysis

A

haptoglobin

96
Q

Which Ig works best at 37C?

97
Q

Macrophages in the spleen, liver, and marrow are a part of ________ hemolysis

A

Extravascular

98
Q

In G6PD deficiency, hemolysis is triggered by ___ or infections

99
Q

What supplement should patients with HA be given?

A

Folate due to rapid turnover of cells

100
Q

In a solution with a tonicity of 0.85%, there will be/ won’t be lysis occurring in someone with hereditary spherocytosis vs. someone who is normal

A

Will be –> test for osmotic fragility

101
Q

Aplastic crisis occurs in patients with hemolytic anemia when they are infected with which pathogen?

A

Parvovirus B19

Hematology Exam 1 (16 decks)

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