Ch. 5 - Normocytic anemia with Intravascular Hemolysis

Question 1

Is PNH congenital or acquired?

Answer 1

acquired

Question 2

What is absent in PNH?

Answer 2

GPI

Question 3

What does GPI do?

Answer 3

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

Question 4

What protects RBCs against complement by inhibiting C3 convertase?

Answer 4

DAF

Question 5

PNH is due to (blank) mediated damage

Answer 5

complement

Question 6

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

Answer 6

once in a while, hence paroxysmal

Question 7

What triggers complement activation in PNH?

Answer 7

shallow breathing during sleep causes mild acidosis leading to complement activation

Question 8

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

Answer 8

HemoglobinemiaHemoglobinuriaHemosiderinuria

Question 9

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

Answer 9

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

Question 10

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

Answer 10

hepatic, portal, or cerebral

Question 11

What complications arise from PNH?

Answer 11

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

Question 12

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

Answer 12

half life

Question 13

What oxidative stress are RBCs routinely exposed to>?

Answer 13

hydrogen peroxide

Question 14

What is needed to reduce oxidized glutathione?

Answer 14

NADPH

Question 15

Which variant of G6PD def. is the mild form?

Answer 15

African

Question 16

Which variant of G6PD def. is the severe form?

Answer 16

Mediterranean

Question 17

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

Answer 17

protection against malaria falciparum

Question 18

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

Answer 18

Heinz bodies

Question 19

What are some notable causes of oxidative stress?

Answer 19

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

Question 20

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

Answer 20

bite

Question 21

G6PD def. causes what type of hemolysis?

Answer 21

intravascular

Question 22

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

Answer 22

back pain and hemoglobinuria

Question 23

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

Answer 23

Heinz stain to screen, enzyme studies to confirm

Question 24

When are enzyme studies done to confirm G6PD def. ?

Answer 24

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

Question 25

IgG mediated autoimmune hemolysis is (warm/cold) agglutination

Answer 25

warm agglutination

Question 26

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

Answer 26

extravascular by the spleen

Question 27

Where in the body does warm agglutination ocurr

Answer 27

in the core ie not the fingers

Question 28

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

Answer 28

spherocytes!

Question 29

What diseases is IgG mediated IHA associated with?

Answer 29

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

Question 30

What are the Tx for IgG mediated IHA?

Answer 30

stop the drug thats causing itIVIGsplenectomy

Question 31

IgM mediated IHA involves (intra/extra)vascular hemolysis

Answer 31

extravascular

Question 32

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

Answer 32

cold; fingers

Question 33

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

Answer 33

C3b is the opsonin leading to spherocytes

Question 34

What leads to intravascular hemolysis in IgM mediated IHA?

Answer 34

extreme activation of complement

Question 35

What two infections can cause IgM mediated IHA?

Answer 35

Mycoplasma pneumonia and infectious mono

Question 36

What is the used to Dx IHA>?

Answer 36

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

Question 37

Direct Coombs test confirms the presence of (blank)

Answer 37

Ab or complement coated RBCs in the pt serum

Question 38

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

Answer 38

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

Question 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

Answer 39

microangiopathic hemolytic anemia

Question 40

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

Answer 40

shistocytes

Question 41

Malaria causes what type of hemolysis?

Answer 41

intravascular

Question 42

T/F: malaria causes extravascular hemolyssi

Answer 42

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

Question 43

Parvovirus b19 infects RBCs and temporarily haults (blank)

Answer 43

erythropoiesis

Question 44

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

Answer 44

marrow stress; ie sickle cell anemia

Question 45

Aplastic anemia presents with (mono/pan)cytopenia

Answer 45

pancytopenia and low RETC

Question 46

What are the causes of aplastic anemia?

Answer 46

drugs, chemicals, viral infections, autoimmune damage

Question 47

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

Answer 47

empty, fatty marrow

Question 48

What drugs are given to correct aplastic anemia?

Answer 48

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

Question 49

Myelophthistic processes present with decreases in what cell lines?

Answer 49

ALL OF THEM