Chapter 30

Question 1

inherited disorders of erythrocytes

__________
disorders are characterized by the presence of an abnormal _______ (__)
mutation causes _____ to replaced by _______
is autosomal ___________

Answer 1

sickle cell disease
hemoglobin
Hb S
valine
glutamic acid
recessive

Question 2

sickle cell disease is classified

disorder initiated by ________ and _______

Answer 2

hypoxemia

acidosis

Question 3

Sickle Cell Disease

Sickling is an ________, ________ phenomenon

• decreased _____ tension (__) of the blood (ex. _______)
• increased _________ concentration in the blood (______)
• increased _________, decreased plasma ________, and low _________

Answer 3

occasional, intermittment
oxygen, PO2, hypoxemia
hydrogen ion, decreased pH
plasma osmolality, volume, temperature

Question 4

sickle cell disease
-___________ crisis (________ crisis)
sickling is in microcirculation, extremely ________ and ________. _______ and _____ exhibit painful swelling (______ syndrome)

this crisis begins with sickling in the microcirculation. As blood flow is ________ by tangled masses of rigid, sickled cells, ______ occurs and a log jam effect brings all blood flow through the vessel to a halt.

Answer 4

vasoocclusive 
thrombotic
painful
symmetric
hands and feet 
hand-foot
obstructed
vasospasm

Question 5

compensatory erythropoeisis is compromised, thus limiting the number of erythrocytes that are replaced

Answer 5

aplastic crisis

Question 6

large amounts of blood pool in the liver and the spleen

Answer 6

sequestrian crisis

Question 7

sickle cell disease
crisis occurs in association with certain drugs or infection
rate of RBC destruction is ___________

Answer 7

hyperhemolytic crisis

accelerated

Question 8

sickled red blood cells attach to the __________ of the injured, underventilated and inflamed _____ and fail to be ________

Answer 8

acute chest syndrome
endothelium
lung
reoxygenated

Question 9

in sickle cell anemia, _______ and __________ are not an option
well matched ______ are often difficult to find

Answer 9

bone marrow
stem cell
donors

Question 10

_______: classic hemophilia, factor ___ deficiency, _______ recessive that affects ____

_______: (christmas disease): factor __ deficiency

Answer 10

hemophilia A
VIII, x-linked
men
hemophilia B
IX