ch 11 pt 2

Question 1

abnormal hemoglobin production, hb is microcytic and hypochromic

Answer 1

thalassemia

Question 2

B-thalassemia involves chromosome __ while a-thalassemia involves chromosome __

Answer 2

11

16

Question 3

a mutation in thalassemia with excess of the opposite globin chain damages RBCs, causing:

Answer 3

hemolysis

Question 4

B-thalassemia ___ involves 1 allele and is asympt or very mild, while B-thalassemia ___ involves 2 alleles and causes splenomegaly, stunted growth, skeletal abnormalities

Answer 4

minor

major

Question 5

treatment of B-thal major that decreases anemia symp and deform and extends survival into 20s

Answer 5

blood transfusions, iron chelation

Question 6

repeated blood transfusions to tx B-thal major can cause eventual iron overload, known as _____, which can cause lethal cardiomyopathy

Answer 6

hemochromatosis

Question 7

a-thalassemia is less severe than B-thal, causes slight decreased O2 capacity, has ineffective ____, and his highly variable due to there being __ total a-globin genes

Answer 7

erythropoiesis

4

Question 8

in a-thal, severity is determined by the # of a-globin gene deletions:

• ___ deletion/s is asymp, or a silent carrier
• ___ deletion/s causes no O2 capacity, and lethal hydrops fetalis

Answer 8

1

4

Question 9

aox that neutralizes oxidants that may cause hemolysis; reduced amounts leaves RBCs oxidative stress

Answer 9

glutathione (GSH)

Question 10

enzyme needed for GSH synth, deficiency of which causes hemolysis
-x linked, MC in males

Answer 10

G6PD (glucose 6 phosphate dehydrogenase)

Question 11

G6PD deficiency is typically asymptomatic until the pt is exposed to an environmental stim that creates oxidative stress such as (2):

Answer 11

infections (MC)
ADRs
(fava beans)

Question 12

a pt with fatigue, pale skin, splenomegaly, back/abdom pain, and hemosiderinuria that occured in acute onset is most likely to have:

Answer 12

G6PD deficiency

Question 13

2 tx for G6PD deficiency, depending on severity

Answer 13

terminate provocative stim
blood transfusion (partial splenectomy)

Question 14

acquired mutation in myeloid stem cells that increases complement fixation onto RBCs and causes hemolysis
-decreases pH while sleeping, dark urine upon waking

Answer 14

PNH (paroxysmal nocturnal hemoglobinuria)

Question 15

tx for PNH involves antibodies that inhibit the:

Answer 15

MAC

Question 16

____ anemias are rare, and involve RBCs being attacked by the immune system, causing opsonization and complement fixation of/onto rbc’s

Answer 16

immunohemolytic

Question 17

two causes of immuno hemolytic anemia:

-____ which is spontaneous and idiopathic, or ____, caused by ADRs, toxic exposure

Answer 17

endogenous

exogenous

Question 18

WARM antibody immunohemolytic anemias function at ___ and involve ____, and are MC idiopathic (primary)
COLD antibody immunohemolytic anemias function at

Answer 18

98.6* F
IgG
86* F
IgM

Question 19

immunohemolytic anemias have subtle features such as chronic/mild anemia, ___, or __ ___

Answer 19

jaundice

raynaud phenomenon

Question 20

sign of repetitive physical stress to RBCs, such as prosthetic heart valves or narrowing of vasc

Answer 20

traumatic hemolysis

Question 21

hemolysis caused by the narrowing of vasculature is called ____ hemolytic anemia

Answer 21

microangiopathic

Question 22

traumatic hemolysis damages RBCs, creating fragmented rbcs called ___, which should prompt investigation into causation

Answer 22

schistocytes

Question 23

more lethal form of malaria is from ___ ___, from the female anopheles mosquito and cause merozoite showers (fever) every ___ hours

Answer 23

plasmodium falciparum

48

Question 24

a pt with: episodic shaking, chills and fevers , vomiting, sweating, hemoglobinuria, renal failure, jaundice, splenomegaly, HA, joint pain may have ____!

Answer 24

malaria

Question 25

___ malaria has cns involvement and may be lethal within days due to RENAL failure

Answer 25

cerebral