Chapter 11 Part II (QUIZ)

Question 1

Sickle cell anemia is autosomal ______ with an mutation/instability of the _____ globin

Answer 1

recessive

B-globin

Question 2

8% of African Americans are ______ or _____

Answer 2

heterozygous carriers

Question 3

sickling –> _______ obstruction

Answer 3

microvascular obstruction

Question 4

locations of sickle cell anemia occurs in areas of _____; bone marrow, spleen; usually has symptoms of _____ and _____/inflammation

Answer 4

stasis
dehydration
infection

Question 5

thrombosis, chronic low-level pain, joint pain, priapism, fever, malaise, splenomegaly/infarction, gallstones are symptoms of

Answer 5

sickle cell anemia

Question 6

heterozygous carriers for sickle cell anemia are RARELY _____ ; homozygous: ___% survive beyond 5th decade

Answer 6

symptomatic

50%

Question 7

MC cause of death in someone with SCA (2)

Answer 7

acute chest syndrome

stroke

Question 8

MC location of congestion/infarction for SCA

Answer 8

bone marrow

Question 9

vertebral involvement in SCA is called _______; microvascular endplate infarctions with central endplate depression

Answer 9

Lincoln log vertebra (H-shaped vertebra)

Question 10

thalassemia is autosomal ________ with abnormal ______ PRODUCTION

Answer 10

recessive

hemoglobin

Question 11

hemoglobin shape in thalassemia (2)

Answer 11

microcytic (small)

hypochromic (pale)

Question 12

areas of endemic malaria (2)

Answer 12

africa

SE asia

Question 13

B-thalassemia is on chromosome ____

Answer 13

11

Question 14

A-thalassemia is on chromosome ___

Answer 14

16

Question 15

mutation in thalassemia is excess of the ________ globin chain

Answer 15

opposite

Question 16

in thalassemia, there is damage to _____ called HEMOLYSIS and damage to ____

Answer 16

RBCs

erythroblasts

Question 17

in ______ thalassemia, there is decreased B-globin chain synthesis and excess of unpaired A chains

Answer 17

B-thalassemia

Question 18

aka B-thalassemia trait; features are ASYMPTOMATIC or MILD; normal life span of RBC; treatment is often not necessary

Answer 18

B-thalassemia minor

Question 19

type of B-thalassemia that has severe hemolysis/anemia with SKELETAL DEFORMITIES; leads to eventual IRON OVERLOAD

Answer 19

b-thalassemia major

Question 20

the eventual iron OVERLOAD in b-thalassemia is called _______ and leads to lethal ______

Answer 20

hemochromatosis

cardiomyopathy

Question 21

a-thalassemia involves ____ a-globin gene, and _____ B-globin

Answer 21

mutated

excess

Question 22

a-thalassemia is ____ severe than b-thalassemia

Answer 22

less

Question 23

__-thalassemia is HIGHLY variable, with __ total globin chains

Answer 23

a-thalassemia

4 (higher # = more severe)

Question 24

function is to neutralize oxidants that may cause hemolysis

Answer 24

glutathione (GSH)

Question 25

enzyme needed for GSH synthesis

Answer 25

G6PD

Question 26

a type of hemolytic anemia in which a deficiency --> DECREASE GSH --> HEMOLYSIS

Answer 26

glucose 6-phosphate dehydrogenase deficiency (G6PD)

Question 27

MC method of decrease GSH production is

Answer 27

deficiency of G6PD

Question 28

who is most affected with G6PD?

Answer 28

males (x-linked)

Question 29

MC environmental stimulus that creates oxidative stress with G6PD (asymptomatic until environmental stimulus)

Answer 29

infection | others are ADRs and fava beans

Question 30

oxidation in G6PD is associated with _____ PHAGOCYTOSIS

Answer 30

splenic

Question 31

splenic macrophages that are due to loss of deformability

Answer 31

bite cells (G6PD)

Question 32

oxidized Hb = clumps; damages RBC membrane --> hemolysis

Answer 32

Heinz bodies (G6PD)

Question 33

acute onset, fatigue, pale skin, splenomegaly, back of abdominal pain, hemosideruria; hemolysis 2-3 days after exposure

Answer 33

G6PD

Question 34

treatment of G6PD depends on ______; identify/______ provocative stimulus, possible _____ or partial splenectomy

Answer 34

severity terminate blood transfusion

Question 35

rare type of hemolytic anemia that is ACQUIRED in MYELOID stem cells

Answer 35

paroxysmal nocturnal hemoglobinuria (PNH)

Question 36

gene involved in PNH

Answer 36

PIGA

Question 37

in paroxysomal noctural hemoglobinuria, there is _____ complement fixation onto RBC's, also ____ urine UPON WAKING because of ______ while sleeping

Answer 37

increase dark decrease pH

Question 38

increase of thrombosis and ___ anemia is MC with PNH

Answer 38

mild

Question 39

treatment for PNH

Answer 39

antibodies that inhibit the MAC

Question 40

type of hemolytic anemia in which antibodies bind RBCs; rare | *chronic/mild anemia and Raynaud phenomenon

Answer 40

immunohemolytic anemias

Question 41

in immunohemolytic anemias, there is ______ of RBCs and complement fixation onto RBCs

Answer 41

opsonization

Question 42

more immunohemolytic anemias are _____ (IDIOPATHIC and SPONTANEOUS) than ______ (ADRs, toxic exposure)

Answer 42

endogenous | exogenous

Question 43

_____ antibody immunohemolytic anemias are at 98.6 F and antibody is ____

Answer 43

warm | IgG

Question 44

____ antibody immunohemolytic anemias are at

Answer 44

cold | IgM

Question 45

hemolytic anemia with sign of repetitive physical stress to RBCs

Answer 45

traumatic hemolysis

Question 46

traumatic hemolysis can occur incidentally during any activity, but more common with ________ and ______

Answer 46

prosthetic heart valves | narrowing of vasculature

Question 47

hemolytic anemia that is observed in pathologic states in which small vessels become partially obstructed or narrowed by lesion that predispose passing red cells to mechanical damage

Answer 47

microangiopathic hemolytic anemia

Question 48

morphologic alterations in injured RBCs are called

Answer 48

schistocytes

Question 49

(2) alterations in schistocytes

Answer 49

burr cells | helmet cells