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Hemolytic Anemia Flashcards

1
Q

The basic pathophysiology of HS is that spectrin, ankyrin, or band 3 defects weaken the _____ and destabilize the ______.

A

cytoskeleton; lipid bilayer

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2
Q

______ are more susceptible in vitro to osmotic stress, the basis for a common test for the disorder.

A

Spherocytes

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2
Q

What happens when the cold antibodies and RBC move centrally to areas of higher temperature?

A

the Ab dissociates b/c of low affinity and complement destroys the RBC (intravascular hemolysis)

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3
Q

The basic pathophysiology of HS is that ____, _____, or _____ weaken the cytoskeleton and destabilize the lipid bilayer.

A

spectrin; ankyrin; band 3 defects

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3
Q

By definition, autoimmune hemolytic anemia should have a _____.

A

positive DAT

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3
Q

What does the AIHA smear look like?

A

spherocytes, teardrop cells, bite cells

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4
Q

The tetramer form of _____ is unstable and dissociates into αβ dimers which may immediately bind to haptoglobin.

A

hemoglobin

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4
Q

What are 2 complications of HS?

A

1) aplastic crisis 2) bilirubin stones

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4
Q

What is the treatment for G6PD?

A

none- just avoid oxidant drugs and foods; treat anemia as needed

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5
Q

How is G6PD inherited?

A

X-linked recessive

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6
Q

Cold antibodies bound to the RBC activate ______ through the C5-C9 attack complex, which creates holes in the plasma membrane.

A

complement

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7
Q

In ______, phosphoenolpyruvate cannot be converted to pyruvate, so ATP is decreased, 2,3-DPG is increased, membrane plasticity is reduced, and the spleen destroys the RBCs

A

pyruvate kinase (PK) deficiency

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8
Q

How do you prevent sepsis in pts with a splenectomy?

A

immunizations against the flu and pneumonia; prophylactic antibiotics

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9
Q

What is the method of inheritance of HS?

A

75% auto dominant; 25% recessive

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9
Q

Defects in the RBC _____ and associated ____ pathways may present with hemolytic anemia.

A

glycolytic; enzyme

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10
Q

The tetramer form of hemoglobin is unstable and dissociates into αβ dimers which may immediately bind to _____.

A

haptoglobin

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10
Q

What does G6PD protect against?

A

oxidant stress

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11
Q

The unconjugated bilirubin fraction will be _____ in hemolytic anemia.

A

elevated

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11
Q

By definition, _____ should have a positive DAT.

A

autoimmune hemolytic anemia

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12
Q

What morphologic features of RBCs are seen in G6PD?

A

bite cells, microspherocytes

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13
Q

Defects in the RBC glycolytic and associated enzyme pathways may present with ______.

A

hemolytic anemia

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13
Q

At what temperature is maximal effect of warm hemolytic anemia seen?

A

37 deg C

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14
Q

Why is splenectomy contraindicated in children under 5?

A

it is important for development of the adaptive humoral response and is the origin of IgM agglutinins, especially for encapsulated organisms

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15
Q

_____ is characterized by the acute or chronic onset of anemia, pallor, jaundice, and dark urine.

A

Autoimmune hemolytic anemia (AIHA)

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15
Autoimmune hemolytic anemia (AIHA) is characterized by the acute or chronic onset of \_\_\_\_, \_\_\_\_, \_\_\_\_, and \_\_\_\_\_.
anemia; pallor; jaundice; dark urine
17
How will the metheme or methemalbumin appear in the CBC in hemolytic anemia?
increased
17
G6PD is associated with selective resistance to \_\_\_\_\_.
plasmodium vivax/malaria
18
What is the most common glycolytic enzyme defect affecting RBCs?
pyruvate kinase (PK) deficiency
19
What is the rate of sepsis in splenectomy pts as compared to the general population?
200x
20
What 2 enzymes may be elevated in hemolytic anemia after being spilled by RBCs?
SGOT and LDH
21
What tests should be run to check for IgG and/or complement on the RBCs?
antiglobulin or Coombs tests
22
How are RBCs bound by warm antibodies destroyed?
antibody-mediated phagocytosis via the splenic macs (extravascular hemolysis)
24
The hemoglobin/haptoglobin complex is removed from circulation by the \_\_\_\_.
liver
25
Cold antibodies bound to the RBC activate complement through the \_\_\_\_\_\_, which creates holes in the plasma membrane.
C5-C9 attack complex
26
What kind of antibodies are seen in warm hemolytic anemia?
IgG
28
What cells in the spleen remove the fragile RBCs?
macs
29
Red cells undergoing _____ release hemoglobin into the circulation.
intravascular hemolysis
29
What are the 2 kinds of autoimmune hemolytic anemia?
cold and warm
31
The tetramer form of hemoglobin is unstable and dissociates into ____ which may immediately bind to haptoglobin.
αβ dimers
32
Red cells undergoing intravascular hemolysis release _____ into the circulation.
hemoglobin
33
The symptoms of HS can be variable, so what is the hallmark of the disorder?
loss of PM and formation of the microspherocyte
35
With \_\_\_\_\_, the red cell is ingested by macrophages of the reticuloendothelial (RE) system.
extravascular hemolysis
37
Hereditary spherocytosis (HS) is a familial disorder characterized by \_\_\_\_, \_\_\_\_\_, \_\_\_\_\_, and \_\_\_\_\_.
anemia, intermittent jaundice, splenomegaly, responsiveness to splenectomy
38
\_\_\_\_ is associated with selective resistance to plasmodium vivax/malaria.
G6PD
38
What happens in pyruvate kinase (PK) deficiency?
phosphoenolpyruvate cannot be converted to pyruvate, so ATP is decreased, 2,3-DPG is increased, membrane plasticity is reduced, and the spleen destroys the RBCs
39
What are the treatments for HS?
supportive care, transfusions, splenectomy
41
What are milder syndromes assoc. with defects in the RBC cytoskeleton?
1) hereditary elliptocytosis 2) stomatocytosis 3) acanthocytosis 4) echinocytosis
42
The _____ is removed from circulation by the liver.
hemoglobin/haptoglobin complex
44
What is aplastic crisis?
when a viral infection causes severe anemia bc of suppressed erythropoiesis coupled to short RBC lifespan
45
Spherocytes are more susceptible in vitro to \_\_\_\_, the basis for a common test for the disorder.
osmotic stress
46
With extravascular hemolysis, the RBC is ingested by _____ of the reticuloendothelial (RE) system.
macrophages
47
Loss of membrane and formation of the spherocyte leads to decreased _____ and \_\_\_\_\_\_.
deformability; entrapment in the spleen
48
What intracellular disorders can result in shortened RBC lifespans?
defects in the plasma membrane/cytoskeleton, enzyme disorders, and hemoglobinopathies
50
How will the serum haptoglobin levels appear on the CBC in hemolytic anemia?
decreased
51
What kind of antibodies are seen in cold hemolytic anemia?
IgG and IgM
53
Hemoglobin in the plasma or urine indicates \_\_\_\_\_.
intravascular hemolysis
56
What happens in G6PD deficient RBCs?
they are unable to restore reduced glutathione, which deforms the membrane
57
On the CBC, what bilirubin test will be elevated in hemolytic anemia?
the unconjugated bilirubin fraction
58
At what temperature is maximal effect of cold hemolytic anemia seen?
4 deg C
60
With extravascular hemolysis, the red cell is ingested by macrophages of the \_\_\_\_\_.
reticuloendothelial (RE) system
61
What cell morphology will be seen in PKD?
nothing specific
62
What is the most common infection seen in pts post splenectomy?
S. pneumoniae
63
How are RBCs bound by cold antibodies destroyed?
intravascular hemolysis
64
Why are bilirubin stones common in HS?
large amounts of bilirubin circulates
65
\_\_\_\_\_ bound to the RBC activate complement through the C5-C9 attack complex, which creates holes in the plasma membrane.
Cold antibodies
66
\_\_\_\_\_ is a familial disorder characterized by anemia, intermittent jaundice, splenomegaly, and responsiveness to splenectomy.
Hereditary spherocytosis (HS)
67
What are the 2 mechanisms of RBC destruction?
1) intravascular 2) extravascular

Blood and Lymph (24 decks)

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