Ch. 5 - Normocytic anemia with Intravascular Hemolysis Flashcards Preview

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Flashcards in Ch. 5 - Normocytic anemia with Intravascular Hemolysis Deck (49):
1

Is PNH congenital or acquired?

acquired

2

What is absent in PNH?

GPI

3

What does GPI do?

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

4

What protects RBCs against complement by inhibiting C3 convertase?

DAF

5

PNH is due to (blank) mediated damage

complement

6

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

once in a while, hence paroxysmal

7

What triggers complement activation in PNH?

shallow breathing during sleep causes mild acidosis leading to complement activation

8

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

HemoglobinemiaHemoglobinuriaHemosiderinuria

9

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

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

10

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

hepatic, portal, or cerebral

11

What complications arise from PNH?

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

12

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

half life

13

What oxidative stress are RBCs routinely exposed to>?

hydrogen peroxide

14

What is needed to reduce oxidized glutathione?

NADPH

15

Which variant of G6PD def. is the mild form?

African

16

Which variant of G6PD def. is the severe form?

Mediterranean

17

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

protection against malaria falciparum

18

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

Heinz bodies

19

What are some notable causes of oxidative stress?

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

20

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

bite

21

G6PD def. causes what type of hemolysis?

intravascular

22

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

back pain and hemoglobinuria

23

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

Heinz stain to screen, enzyme studies to confirm

24

When are enzyme studies done to confirm G6PD def. ?

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

25

IgG mediated autoimmune hemolysis is (warm/cold) agglutination

warm agglutination

26

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

extravascular by the spleen

27

Where in the body does warm agglutination ocurr

in the core ie not the fingers

28

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

spherocytes!

29

What diseases is IgG mediated IHA associated with?

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

30

What are the Tx for IgG mediated IHA?

stop the drug thats causing itIVIGsplenectomy

31

IgM mediated IHA involves (intra/extra)vascular hemolysis

extravascular

32

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

cold; fingers

33

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

C3b is the opsonin leading to spherocytes

34

What leads to intravascular hemolysis in IgM mediated IHA?

extreme activation of complement

35

What two infections can cause IgM mediated IHA?

Mycoplasma pneumonia and infectious mono

36

What is the used to Dx IHA>?

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

37

Direct Coombs test confirms the presence of (blank)

Ab or complement coated RBCs in the pt serum

38

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

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

39

(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

microangiopathic hemolytic anemia

40

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

shistocytes

41

Malaria causes what type of hemolysis?

intravascular

42

T/F: malaria causes extravascular hemolyssi

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

43

Parvovirus b19 infects RBCs and temporarily haults (blank)

erythropoiesis

44

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

marrow stress; ie sickle cell anemia

45

Aplastic anemia presents with (mono/pan)cytopenia

pancytopenia and low RETC

46

What are the causes of aplastic anemia?

drugs, chemicals, viral infections, autoimmune damage

47

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

empty, fatty marrow

48

What drugs are given to correct aplastic anemia?

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

49

Myelophthistic processes present with decreases in what cell lines?

ALL OF THEM