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All the things continued > 5.6 and 5.7 Intravascular hemolysis and underproduction > Flashcards

5.6 and 5.7 Intravascular hemolysis and underproduction Flashcards

1
Q

What are the two components on RBCs that inhibit complement activation?

A

MIRL

DAF (CD55)

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2
Q

What is the function of DAF?

A

Accelerates the decay of C3 convertase

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3
Q

What is the anchoring protein found on RBCs that allow for DAF and MIRL to stick?

A

GpI

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4
Q

What is the cause of PNH?

A

Acquired defect in myeloid stem cells, that deletes the GpI anchor on RBCs, allowing RBCs to be destroyed by complement

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5
Q

What accounts for the paroxysmal part of PNH?

A

Shallow breathing at night causes a mild respiratory acidosis, leading to the activation of complement

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6
Q

What type of hemolysis occurs with PNH? What, then, are the common s/sx?

A

Intravascular:

  • Hemoglobinemia
  • Hemoglobinuria
  • Hemosiderinuria
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7
Q

What is the screening test for PNH, and how does it work?

A

Introduce sucrose into the serum, which activates complement

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8
Q

What is the confirmatory test for PNH?

A

Acidified serum test or flow cytometry for a lack of CD55

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9
Q

What is the main cause of death in patients with PNH? How does this occur?

A

Thrombosis (e.g. of hepatic, portal, or cerebral veins)

-Destroyed platelets release cytoplasmic contents into circulation, inducing thrombosis

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10
Q

What causes the Fe deficiency anemia that occurs with PNH?

A

Loss of Fe in urine

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11
Q

What is the cancer that occurs with PNH?

A

AML due to mutation in myeloid stem cell

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12
Q

What is the inheritance pattern with G6PD deficiency?

A

XLR

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13
Q

What is the molecule that reduces the H2O2 and other free radicals in RBCs?

A

Glutathione

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14
Q

What is the pathophysiology behind G6PD deficiency?

A

Reduced half life of G6PD prevent the reduction of NADP to NADPH, meaning that glutathione cannot be reduced to prevent RBCs from being damaged from oxidative stress

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15
Q

What are the two variants of G6PD deficiency?

A
  • African = mildly reduced half life of G6PD

- Mediterranean = markedly reduced half life of G6PD

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16
Q

What is the thinking behind the preponderance of G6PD deficiency?

A

Protective effect against falciparum malaria

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17
Q

What are the causes of oxidative stress that classically precipitate G6PD episodes?

A
  • Fava beans
  • Sulfa drugs
  • Primaquine
  • Dapsone
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18
Q

What is the classic PBS finding in G6PD deficiency? Why?

A

Bite cells from removal of Heinz bodies

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19
Q

What type of hemolysis occurs with G6PD deficiency?

A

Intravascular

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20
Q

What happens to Hb when it is exposed to oxidative stress?

A

Precipitates as a Heinz body

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21
Q

What are the classic s/sx of G6PD deficiency?

A

Hemoglobinuria and back pain hours after exposure to an oxidative stress

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22
Q

What is the screening and confirmatory test for G6PD deficiency?

A
  • Screen = Heinz prep

- Confirmatory = enzyme studies

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23
Q

What are the antibody classes that cause immune hemolytic anemia?

A

IgG or IgM

24
Q

IgG mediated immune hemolytic anemia usually results in intra or extravascular hemolysis? Why?

A

Extravascular, since

25
Q

Why should you never perform an enzyme test for immune hemolytic anemia during an acute attack?

A

Cells that lack the enzyme will be destroyed, and thus will result in a false negative test

26
Q

IgG binds to RBCs centrally or peripherally in immune hemolytic anemia? IgM?

A 
IgG = centrally
IgM = peripherally
27
Q

What happen to RBCs with immune hemolytic anemia? Why?

A

Spherocytosis due to a slow loss of membrane

28
Q

What are the two major diseases in which warm agglutinins occurs?

A

SLE

CLL

29
Q

How does PCN lead to immune hemolytic anemia?

A

Drug attaches to RBC membrane, allowing for IgG to attach to and destroy RBCs

30
Q

How does methyldopa lead to immune hemolytic anemia?

A

Drug induces immune antibodies itself

31
Q

What is the treatment for immune hemolytic anemia? (4)

A
  • Cessation of drug
  • Steroid
  • IVIG
  • Splenectomy
32
Q

Cold agglutinins (IgM mediated immune hemolytic anemia) is associated with what infectious diseases?

A
  • Mycoplasma pneumoniae

- EBV

33
Q

What is the diagnostic test for immune hemolytic anemia?

A

Coombs test

34
Q

What is the direct coombs test?

A

Patient’s RBCs added to anti-IgG antibody. Will agglutinate if RBCs are already coated with IgG

35
Q

What is the indirect coombs test?

A

Take normal RBCs and add to patient’s serum.

Anti-IgG is then added to test for the presence of ab-RBC complexes

36
Q

What are the thrombi composed of with TTP?

A

Platelets

37
Q

What is the enzyme that is deficient in TTP? What does this cause?

A

ADAMTS13- prevents vWF polymer from being broken down into monomers, resulting in

38
Q

What are the thrombi composed of in DIC?

A

Platelet and fibrin

39
Q

What is the characteristic PBS finding of microangiopathic anemia?

A

Schistocytes

40
Q

What are the heart pathologies that may lead to schistocyte formation?

A
  • Prosthetic heart valves

- Aortic stenosis

41
Q

What is the characteristic PBS findings of plasmodium falciparum?

A

Banana

42
Q

What is the characteristic PBS finding of babesiosis?

A

Maltese cross

43
Q

What is the vector for malaria?

A

Anopheles mosquito

44
Q

What causes the s/sx of malaria?

A

Intravascular hemolysis of RBC that occurs as a part of plasmodium life cycle

45
Q

What is the species of plasmodium that causes daily fevers?

A

Falciparum

46
Q

What is the species of plasmodium that causes fevers every other day? What is the treatment?

A

Vivax and ovale

Praziquantel

47
Q

What is the diagnostic finding of anemia 2/2 underproduction?

A

Low corrected reticulocyte count

48
Q

What are the three major etiologies of anemia 2/2 underproduction?

A
  • Macro or microcytic causes
  • Renal failure
  • Bone marrow precursor cell damage
49
Q

What is the treatment for underproduction anemia 2/2 parvovirus B19 infection?

A

Supportive

50
Q

What is the cause of aplastic anemia?

A

Damage to HSC, resulting in a pancytopenia

51
Q

What is the CD marker for hematopoietic cells?

A

CD34

52
Q

What antipsychotic drug classically causes agranulocytosis?

A

Clozapine

53
Q

What are the common etiologies of aplastic anemia?

A

Drugs
Viruses
Autoimmune damage

54
Q

What is the classic bone marrow aspirate finding of aplastic anemia?

A

dry tap–fat in bone

55
Q

What is the treatment for aplastic anemia, besides obviously d/c drugs that cause it? (4)

A
  • Transfusions
  • Marrow stimulating factors
  • Immunosuppression
  • BMT
56
Q

What is a myelophthisic process?

A

Pathologic process that replaces bone marrow. This impairs hematopoiesis, and resulting in a pancytopenia

All the things continued (28 decks)

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