Chapter 11 Part II (QUIZ) Flashcards

1
Q

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

A

recessive

B-globin

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2
Q

8% of African Americans are ______ or _____

A

heterozygous carriers

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3
Q

sickling –> _______ obstruction

A

microvascular obstruction

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4
Q

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

A

stasis
dehydration
infection

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5
Q

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

A

sickle cell anemia

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6
Q

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

A

symptomatic

50%

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7
Q

MC cause of death in someone with SCA (2)

A

acute chest syndrome

stroke

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8
Q

MC location of congestion/infarction for SCA

A

bone marrow

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9
Q

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

A

Lincoln log vertebra (H-shaped vertebra)

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10
Q

thalassemia is autosomal ________ with abnormal ______ PRODUCTION

A

recessive

hemoglobin

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11
Q

hemoglobin shape in thalassemia (2)

A

microcytic (small)

hypochromic (pale)

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12
Q

areas of endemic malaria (2)

A

africa

SE asia

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13
Q

B-thalassemia is on chromosome ____

A

11

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14
Q

A-thalassemia is on chromosome ___

A

16

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15
Q

mutation in thalassemia is excess of the ________ globin chain

A

opposite

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16
Q

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

A

RBCs

erythroblasts

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17
Q

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

A

B-thalassemia

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18
Q

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

A

B-thalassemia minor

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19
Q

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

A

b-thalassemia major

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20
Q

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

A

hemochromatosis

cardiomyopathy

21
Q

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

A

mutated

excess

22
Q

a-thalassemia is ____ severe than b-thalassemia

A

less

23
Q

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

A

a-thalassemia

4 (higher # = more severe)

24
Q

function is to neutralize oxidants that may cause hemolysis

A

glutathione (GSH)

25
Q

enzyme needed for GSH synthesis

A

G6PD

26
Q

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

A

glucose 6-phosphate dehydrogenase deficiency (G6PD)

27
Q

MC method of decrease GSH production is

A

deficiency of G6PD

28
Q

who is most affected with G6PD?

A

males (x-linked)

29
Q

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

A

infection

others are ADRs and fava beans

30
Q

oxidation in G6PD is associated with _____ PHAGOCYTOSIS

A

splenic

31
Q

splenic macrophages that are due to loss of deformability

A

bite cells (G6PD)

32
Q

oxidized Hb = clumps; damages RBC membrane –> hemolysis

A

Heinz bodies (G6PD)

33
Q

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

A

G6PD

34
Q

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

A

severity
terminate
blood transfusion

35
Q

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

A

paroxysmal nocturnal hemoglobinuria (PNH)

36
Q

gene involved in PNH

A

PIGA

37
Q

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

A

increase
dark
decrease pH

38
Q

increase of thrombosis and ___ anemia is MC with PNH

A

mild

39
Q

treatment for PNH

A

antibodies that inhibit the MAC

40
Q

type of hemolytic anemia in which antibodies bind RBCs; rare

*chronic/mild anemia and Raynaud phenomenon

A

immunohemolytic anemias

41
Q

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

A

opsonization

42
Q

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

A

endogenous

exogenous

43
Q

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

A

warm

IgG

44
Q

____ antibody immunohemolytic anemias are at

A

cold

IgM

45
Q

hemolytic anemia with sign of repetitive physical stress to RBCs

A

traumatic hemolysis

46
Q

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

A

prosthetic heart valves

narrowing of vasculature

47
Q

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

A

microangiopathic hemolytic anemia

48
Q

morphologic alterations in injured RBCs are called

A

schistocytes

49
Q

(2) alterations in schistocytes

A

burr cells

helmet cells