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Pathoma LAH > RBCs: 5.6: Normocytic w/ Intravascular Hemolysis > Flashcards

RBCs: 5.6: Normocytic w/ Intravascular Hemolysis Flashcards

1
Q

What causes paroxysmal nocturnal hemoglobinuria?

A

an acquired defect of GPI, an anchoring protein for protective surface proteins against complement, on blood cells

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2
Q

What destroys the cells in paroxysmal nocturnal hemoglobinuria?

A

complement

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3
Q

Is the hemolysis in paroxysmal nocturnal hemoglobinuria intra- or extravascular?

A

intravascular

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4
Q

Shallow breathing leads to what acid-base disturbance?

A

respiratory acidosis

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5
Q

What are the lab findings of paroxysmal nocturnal hemoglobinuria?

A

hemoglobinemia
hemoglobinuria
hemosiderinuria (days later)

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6
Q

How do you screen for paroxysmal nocturnal hemoglobinuria?

A

give sucrose- activate complement- positive = cell destruction

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7
Q

How do you confirm paroxysmal nocturnal hemoglobinuria?

A

lack of CD55 (DAF) on RBCs or the acidified serum test

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8
Q

What is the main cause of death in paroxysmal nocturnal hemoglobinuria?

A

thrombosis (platelet destruction activates coag cascade)

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9
Q

What are some complications of paroxysmal nocturnal hemoglobinuria?

A

Fe deficiency anemia (lost in urine)

AML (bc already have 1 mutation in myeloid stem cells)

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10
Q

How is G6PD inherited?

A

X-linked

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11
Q

What does glutathione do?

A

protects RBCs against oxidative stress

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12
Q

What reduces glutathione?

A

NADPH

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13
Q

What produces NADPH in an RBC?

A

G6PD

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14
Q

What is the underlying problem in G6PD?

A

a reduced half-life of G6PD –> increased suscept. to oxidative stress in RBCs

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15
Q

What are the 2 variants of G6PD?

A
  1. African (mild)

2. Mediterranean (moderate)

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16
Q

How long is the G6PD half life in a normal pt?

A

as long as the RBC’s life

17
Q

Name 2 diseases that are protective for falciparum malaria.

A
  1. sickle cell

2. G6PD

18
Q

Name 3 drugs that cause oxidative stress to RBCs.

A
  1. primaquine
  2. sulfa drugs
  3. dapsone
19
Q

Oxidative stress in RBCs precipitates hemoglobin into a ____.

A

Heinz body

20
Q

How does a G6PD crisis present?

A

hemoglobinuria, back pain (nephrotoxic) within hours of exposure

21
Q

What is the screening test for G6PD?

A

a Heinz preparation (highlights precipitated Hb)

22
Q

What is the confirmatory test for G6PD?

A

enzyme studies after the acute episode has resolved

23
Q

What is immune hemolytic anemia?

A

Ab mediated (IgG or IgM) destruction of RBCs

24
Q

What kind of Ab causes immune hemolytic anemia?

A

IgG or IgM

25
Q

What happens to IgG covered RBCs in immune hemolytic anemia?

A

the spleen macs eat the membrane –> spherocytes

26
Q

Name 2 diseases associated with immune hemolytic anemia.

A

SLE, CLL

27
Q

Name 2 drugs that can cause immune hemolytic anemia.

A
  1. penicillin

2. methyldopa

28
Q

What is the tx for immune hemolytic anemia?

A
  1. stop offending drug, if applicable
  2. steroids
  3. IVIG (distraction technique)
  4. splenectomy
29
Q

At what temperature does IgM immune hemolytic anemia prefer?

A

cold temps

30
Q

Name 2 diseases that IgM immune hemolytic anemia are seen in.

A

Mycoplasma pneumo

mononucleosis

31
Q

How is immune hemolytic anemia diagnosed?

A

Coombs test (esp direct)

32
Q

How does a direct Coombs test work?

A

confirms the presence of Ab-coated RBCs (anti-IgG added- positive if another Ab already existed)

33
Q

How does a indirect Coombs test work?

A

it checks for Abs in the serum (give test RBCs to see if agglutination occurs when anti-IgG is added)

34
Q

What is microangiopathic hemolytic anemia?

A

when RBCs are destroyed as they pass thru BVs with platelet microthrombi on the sidewalls

35
Q

What cells result from microangiopathic hemolytic anemia?

A

shistocytes

36
Q

Name 5 syndromes that cause shistocytes.

A
  1. DIC
  2. HUS
  3. TTP
  4. HELLP
  5. microangiopathic hemolytic anemia
37
Q

What is another name for a shistocyte?

A

helmet cell

Pathoma LAH (77 decks)

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