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PATHOMA > Ch. 5 - Normocytic anemia with Intravascular Hemolysis > Flashcards

Ch. 5 - Normocytic anemia with Intravascular Hemolysis Flashcards

1
Q

Is PNH congenital or acquired?

A

acquired

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2
Q

What is absent in PNH?

A

GPI

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3
Q

What does GPI do?

A

connects DAF and MIRL to the cell membrane of RBCs, platelets, and WBCs

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4
Q

What protects RBCs against complement by inhibiting C3 convertase?

A

DAF

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5
Q

PNH is due to (blank) mediated damage

A

complement

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6
Q

IN PNH intravascular hemolysis happens (daily/once in a while)

A

once in a while, hence paroxysmal

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7
Q

What triggers complement activation in PNH?

A

shallow breathing during sleep causes mild acidosis leading to complement activation

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8
Q

What two things are seen in the urine and one thing in the blood first thing in the morning with PNH?

A

HemoglobinemiaHemoglobinuriaHemosiderinuria

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9
Q

What is used to screen for PNH? What is used to confirm the Dx?

A

sucrose test used to screen; confirmation is acidified serum test or flow for lack of CD55

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10
Q

Main cause of death in PNH is thrombosis of what three veins?

A

hepatic, portal, or cerebral

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11
Q

What complications arise from PNH?

A

iron def. anemia and AML in 10% of the cases

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12
Q

G6PD def. is an x-linked rec. disorder that causes a reduced (blank) of glutathione

A

half life

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13
Q

What oxidative stress are RBCs routinely exposed to>?

A

hydrogen peroxide

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14
Q

What is needed to reduce oxidized glutathione?

A

NADPH

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15
Q

Which variant of G6PD def. is the mild form?

A

African

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16
Q

Which variant of G6PD def. is the severe form?

A

Mediterranean

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17
Q

What is the reason for the high carrier frequency of G6PD def. in both populations?

A

protection against malaria falciparum

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18
Q

In G6PD def., oxidative stress precipitates Hb as (blank) bodies

A

Heinz bodies

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19
Q

What are some notable causes of oxidative stress?

A

infections, drugs (sulfa and primaquine and dapsone), and fava beans

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20
Q

Removal of Heinz bodies from RBCs by splenic mac’s results in (blank) cells

21
Q

G6PD def. causes what type of hemolysis?

A

intravascular

22
Q

WHat are the two symptoms that normally present a few hours after exposure to oxidative stress?

A

back pain and hemoglobinuria

23
Q

What is used to screen for G6PD def. ? What is used to confirm the Dx?

A

Heinz stain to screen, enzyme studies to confirm

24
Q

When are enzyme studies done to confirm G6PD def. ?

A

weeks after the last episode so the enzyme levels arent depleted.

25
Q

IgG mediated autoimmune hemolysis is (warm/cold) agglutination

A

warm agglutination

26
Q

IgG mediated IHA results in (intra/extra)vascular hemolysis

A

extravascular by the spleen

27
Q

Where in the body does warm agglutination ocurr

A

in the core ie not the fingers

28
Q

What finding on peripheral smear do you see for warm IgG mediated IHA?

A

spherocytes!

29
Q

What diseases is IgG mediated IHA associated with?

A

SLE, CLL, and PCNs that can bind to RBC membrane and be recognized by the IgG as the drug-membrane complex

30
Q

What are the Tx for IgG mediated IHA?

A

stop the drug thats causing itIVIGsplenectomy

31
Q

IgM mediated IHA involves (intra/extra)vascular hemolysis

A

extravascular

32
Q

IgM mediated IHA is (warm/cold) aglutination and happens where in the body?

A

cold; fingers

33
Q

What serves as a opsonin for splenic macs in IgM medated IHA? How does this present on peripheral smear?

A

C3b is the opsonin leading to spherocytes

34
Q

What leads to intravascular hemolysis in IgM mediated IHA?

A

extreme activation of complement

35
Q

What two infections can cause IgM mediated IHA?

A

Mycoplasma pneumonia and infectious mono

36
Q

What is the used to Dx IHA>?

A

Direct or indirect Coomb’s test; direct is the most important

37
Q

Direct Coombs test confirms the presence of (blank)

A

Ab or complement coated RBCs in the pt serum

38
Q

Indrect Coomb’s test confirms the presence of (blank)

A

Abs in the pt serum that will bind to TEST RBCs.

39
Q

(blank) is a process that occurs with diseases like TTP-HUS, DIC, and HELLP, and is an intravascular hemolysis as RBCs pass through small blood vessels with microthrombi

A

microangiopathic hemolytic anemia

40
Q

(blank) cells are seen on peripheral smear with prosthetic heart valves, aortic stenosis, and other causes of microangiopathic hemolytic anemia

A

shistocytes

41
Q

Malaria causes what type of hemolysis?

A

intravascular

42
Q

T/F: malaria causes extravascular hemolyssi

A

true, with splenomegaly. Not as much though as intravascular.

43
Q

Parvovirus b19 infects RBCs and temporarily haults (blank)

A

erythropoiesis

44
Q

Parvovirus b19 infections lead to severe anemia in the setting of what preexisting condition?

A

marrow stress; ie sickle cell anemia

45
Q

Aplastic anemia presents with (mono/pan)cytopenia

A

pancytopenia and low RETC

46
Q

What are the causes of aplastic anemia?

A

drugs, chemicals, viral infections, autoimmune damage

47
Q

A biopsy of someone with aplastic anemia would show what of their bone marrow?

A

empty, fatty marrow

48
Q

What drugs are given to correct aplastic anemia?

A

GM-CSF, G-CSF, immunosppression, and bone marrow transplant

49
Q

Myelophthistic processes present with decreases in what cell lines?

A

ALL OF THEM

PATHOMA (11 decks)

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