Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Hem/Onc > anemia pt 1 > Flashcards

anemia pt 1 Flashcards

1
Q

what makes someone anemic?

A

female
- hgb <12g/dL
- hct <36%
male
- hgb <13.6 g/dL
- hct <41%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

3 major causes of anemia

A
  1. decreased in RBC production
  2. increased destruction of RBC
  3. increased blood loss
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

2 classifications of hemolytic anemia

A
  1. intravascular - RBCs lyse within BLOOD VESSELS
  2. extravascular - RBCs are destroyed within ORGANS
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

what classification of hemolytic anemia would you see:
- large amounts of hgb released into circulation - decrease in haptoglobin; possible hemoglobinuria
- decreased iron over time
- schistocyte formation

A

intravascular hemolytic anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

what classification of hemolytic anemia would you see:
- minimal hgb release in circulation - haptoglobin may or may not decrease
- iron is recovered and stored
- spherocyte formation

A

extravascular hemolytic anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

for extravascular hemolytic anemia, RBC is destroyed in the ?

A

spleen (mostly)
liver

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

PE findings for hemolytic anemia

A

skin - pallor, jaundice
abd - +/- spleen enlargement
urine - red/brown discoloration (intravascular hemolysis)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

lab findings for hemolytic anemias

A

hgb - normal or reduced
MCV - increased
reticulocytes - increased
bilirubin - increased (unconjugated)
LDH - increased
haptoglobin - decreased (intravascular)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

what can falsely elevate haptoglobin lvls

A

inflammatory markers

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

causes of hemolytic anemia

A
  1. structural
    - hereditary spherocytosis
  2. hemoglobinopathies
    - thalassemias
    - sickle cell disease
  3. metabolic
    - G6PD deficiency
  4. immune-related
    - autoimmune hemolytic anemia (AIHA)
    - paroxysmal nocturnal hemoglobinuria (PNH)
    - hemolytic disease of the newborn (HDN)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

cause of hereditary spherocytosis

A

abnormal formation of proteins in the RBC membrane
inherited, mainly autosomal dominant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

pathology of hereditary spherocytosis

A

abnormally shaped RBCs (rounded) = trap in small red pulp fenestrations = phagocytic destruction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

lab findings of hereditary spherocytosis

A

hgb/hct - variably decreased
reticulocytes - increased
MCHC - INCREASED
MCV - normal or low
haptoglobin - normal or low
peripheral blood smear - spherocytes
coombs testing - should be negative

only one that can cause INCREASED MCHC in microcytosis/normocytosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

treatment of hereditary spherocytosis

A
  1. ALL get folic acid
  2. Transfusion - if severe
  3. splenectomy - DEFINITIVE TX
    - moderate - delay until after 5yo or puberty
    - mild - observe
    - antipneumococcal vax
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

3 types of hemoglobin

A
  1. hbA - 97-99% of Hb - “adult” hgb
  2. hbA2 - 1-3%
  3. hbF - <1%
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

chromosome 16 has 2 copies of ?

A

alpha-globulin gene - 4 total

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

chromosome 11 has 1 copy of ?

A

beta-globulin gene - 2 total
also has copies of delta-globulin and gamma-globulin gene

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

cause of alpha thalassemias

A

gene deletion = reduced alpha-chain synthesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

pathology of alpha thalassemias

A
  1. more small, “pale” (low hb) RBCs
  2. excess beta chains precipitate = damaging RBC membrane = hemolysis in marrow and splenic vessels
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

demographics of alpha thalassemias

A

SE asians and chinese descent
mediterranean or african descent

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Hb electrophoresis of alpha thalassemias would present ?

A

normal - equal proportion of hbA, hbA2, hbF

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

lab findings for alpha thalassemias

A

hgb/hct - normal (carrier) or decreased
RBC - increased
MCV - decreased - very tiny
reticulocyte - increased or normal
MCH - decreased
inclusion bodies - in HbH disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

how would the electrophoresis present in alpha thalassemia

A

carriers, alpha talassemia minor - normal
HbH disease - presence of HbH band

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

3 normal alpha-globulin genes result in ?

A

carrier = alpha thalassemia MINIMA
normal lab values (HCT, MCV)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

2 normal alpha globulin genes result in ?

A

alpha thalassemia MINOR
HCT - 28-40%
MCV - 60-75%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

1 normal alpha globulin gene results in ?

A

hemoglobin H disease (HbH)
has 4 beta-chains = little to no use for O2 transport
HCT - 22-32%
MCV - 60-70%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

no normal alpha globulin genes result in ?

A

hydrops fetalis
die in utero

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

describe the peripheral smear findings of alpha thalassemia trait

A
  1. hypochromatic, microcytic cells
  2. target cells
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

describe the peripheral smear findings of HbH

A
  1. hypochromatic, microcytic cells
  2. target cells
  3. poikilocytosis
  4. sometimes some normal RBC bc of transfusions
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

treatment for alpha thalassemia

A
  1. minima
    - no treatment
  2. minor
    - usually no treatment; genetic counseling
    - must monitor iron overload - chelation
  3. HbH
    - folic acid supplement
    - avoid iron supplements and oxidative drugs - MONITOR IRON
    - may need transfusion regularly
    - splenectomy if severe or too frequent transfusions
  4. hydrops fetalis
    - termination
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

cause of beta thalassemias

A

gene point mutation = reduced beta-chain synthesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

types of betathalassemias

A
  1. beta+ - reduced beta-chain synthesis
  2. beta0 - absent beta-chain synthesis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

how would the electrophoresis of beta thalassemias present?

A

increased proportion of HbA2 and HbF
(HbF > HbA2)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

demographics of beta thalassemias

A

mainly mediterranean descent
can be seen in african and asian descent

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

pathology of beta thalassemias

A
  • more small, “pale” (low Hgb) RBC
  • excess alpha chains precipitate = hemolysis in marrow, spleen, liver
  • alpha chains = inclusion bodies = damaged erthyroid precursors (intramedullary)
  • surviving RBCs = inclusion bodies = short lifespan (extramedullary)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

lab findings of beta thalassemia

A

hgb/hct - decreased
RBC - increased
MCV - decreased
reticulocyte - increased
MCH - decreased

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

exam findings of beta thalassemia

A

“chipmunk facies”
thinning of long bones
pathologic fracture
failture to thrive

38
Q

describe the peripheral smear findings of beta thalassemia minor

A

hypochromic, microcytic cells
target cells

39
Q

describe the peripheral smear findings of beta thalassemia intermedia

A

hypochromic, microcytic cells
target cells
poikilocytosis

40
Q

describe the peripheral smear findings of beta thalassemia major

A

hypochromic, microcytic cells
target cells
poikilocytosis
nucleated RBC

41
Q

treatment for beta thalassemia including definitive tx

A
  1. minor
    - no tx; genetic counseling
    - monitor iron overload
  2. intermedia
    - genetic counseling
    - avoid iron supplements - may need if secondary condition causes iron deficiency
    - may require transfusion
    - monitor iron overload
  3. major
    - frq transfusion-dependent
    - avoid iron supplements
    - splenectomy
    - luspatercept - promotes RBC production by interfering with TGF-B signaling
    - bone marrow transplant - ONLY DEFINITIVE TX
42
Q

sickle cell disease is an ____ ____ inherited disease

A

autosomal recessive
does NOT affect a sex more than the other*

43
Q

cause of sickle cell disease

A

abn substitution of an amino acid in the beta chain (betaS)

44
Q

for sickle cell, if you are heterozygous it would be ____, while homozygous would be _____

A

sickle cell trait
sickle cell anemia

45
Q

demographics of sickle cell disease

A

African americans
1 in 400 black children
also mediterranean, latin, and native american descent

46
Q

what % of African Americans make up carriers in the US

A

8%

47
Q

pathology of sickle cell disease

A

unstable HbS polymerizes = sickled shaped = vaso-occlusive episodes
“stick” to endothelium
vasoocclusion = ischemia, pain, end-organ damage

48
Q

what sequesters and destroys sickled RBCs?

A

spleen

49
Q

what triggers sickle cell disease?

A

anything stressful!
hypoxemia, acidosis, infection, excessive exercise, abrupt temp changes, anxiety/stress

50
Q

a pt with sickle cell disease would show what in their hemoglobin electrophoresis?

A

abnormal proportions of HbA, HbA2, HbF
presence of HbS band

51
Q

Howell-Jolly inclusion bodies would be seen in ___

A

sickle cell anemia

52
Q

clinical presentations for sickle cell trait

A

often asymptomatic
can have s/s during physical stress - high altitude, heavy exercise, dehydration

53
Q

when do s/s for sickle cell disease appear?

A

around 6 months old

54
Q

clinical presentations for sickle cell anemia

A
  • poorly healing LE ulcers (10+ y/o)
  • dactylitis
  • vision changes, retina problems
  • splenomegaly (<3 y/o)
  • hyperdynamic precordium
55
Q

clinical presentation of sickle cell crisis

A
  • ischemic injury to 1+ organs
  • sudden pain that lasts from hrs to wks
  • fever, tachycardia
  • MC locations - abd, bones, joints, soft tissues
    – <18m - hands/feet
    – children/teens - long bones of arms/legs
    – adults - vertebrae
56
Q

how do you treat sickle cell anemia/what are the ways

A
  1. avoid triggers
    - hypoxemia
    - temp changes
    - dehydration
    - acidosis
    - stress
  2. supportive
    - Folic acid
    - vaccinations
    - ACE inhibitors
    - omega-3 fatty acids
    - pain management
    - abx = prophylactic PCN daily until age 5, then optional
  3. disease-modifying meds - decreases vasoocclusive episodes
    - hydroxyurea
    - crizanlizumab
    - l-glutamine
57
Q

what is the mainstay medication of sickle cell anemia treatment? what SE must you address

A

hydrourea
bone marrow suppression
- can cause skin ulcers
- increase cancer risk
-teratogenic

58
Q

an acute sickle cell crisis treatment is…

A

HOP - hydration, oxygenation, pain control
- exchange transfusion - vasoocclusive crises, pain crises, other severe sequelae
- splenic sequestration crisis (disprop. amount of blood sequestered in spleen)
– splenectomy to prevent recurrence

59
Q

definitive tx for sickle cell anemia

A

stem cell transplant

60
Q

main cause of G6PD deficiency is…

A

enzyme deficiency
x-linked recessive!!

61
Q

G6PD deficiency is commonly seen in…

A

African american MALES (X-linked recessive)
also in Asian and mediterranean descent

62
Q

what anemia is seen when RBCs are vulnerable to oxidative stress and forms Heinz bodies

A

G6PD deficiency

63
Q

Splenomegaly is usually not seen in G6PD deficient pts because…

A

it is an episodic hemolytic anemia

64
Q

what are the 3 major triggers of G6PD deficiency

A
  1. infections
  2. foods - FAVA BEANS
  3. medications - ABX (sulfas, quinolones, nitrofurantoin), Azo, aspirin
65
Q

dark urine can be seen in G6PD deficiency because …

A

it is INTRAVASCULAR

66
Q

a peripheral smear with bite cells, blister cells, and Heinz bodies is seen in…

A

G6PD deficiency

67
Q

avoiding oxidant drugs is a preventive measure for what anemia

A

G6PD deficiency

68
Q

cause of autoimmune hemolytic anemia

A
  1. antibodies form against RBC antigens (igG)
    - 50% idiopathic
    - diseases - SLE, leukemia, lymphoma
69
Q

autoimmune hemolytic anemia must be distinguished from…

A

drug-induced immune hemolytic anemia
- caused by abx (cephalosporins, PCNs, quinolones), methyldopa or levodopa, or NSAIDs

70
Q

pathology of autoimmune hemolytic anemia

A

RBC tagged for destruction
spherocytes formation

71
Q

what anemia can have complements tag RBC for destruction by Kupffer cells

A

autoimmune hemolytic anemia

72
Q

how does IgM play a role in autoimmune hemolytic anemia

A

facilitates MAC-induced direct intravascular hemolysis

73
Q

what type of hemolytic anemia can be triggered from warm or cold temperatures

A

autoimmune hemolytic anemia

74
Q

difference between direct and indirect Coombs test

A

direct = RBC - reagent mixed with pt RBC
indirect = serum - pt serum is mixed with RBCs; reagent is added

75
Q

marked microspherocytosis can be seen in ?

A

autoimmune hemolytic anemia

76
Q

immunosuppression, comorbidities, and transfusions can be treatments for..

A

autoimmune hemolytic anemia
severe = splenectomy or transfusion

77
Q

cause of hemolytic disease of newborns

A

maternal IgG to antigens on surface of fetal RBC
- fetal-maternal hemorrhage
- hydrops fetalis

78
Q

positive direct Coombs test can be seen on who during hemolytic disease of the newborn

A

newborn
indirect = maternal

79
Q

what is the preventive care for hemolytic disease of newborn

A

RhoGAM - prevents immune response to Rh+ blood in Rh- mother

80
Q

an acquired genetic defect that leads to RBC lysis by complement

A

paroxysmal nocturnal hemoglobinuria

81
Q

Free Hb from NH results in…

A

depleted NO
esophageal spasms, ED, renal damage, thrombosis

82
Q

PNH is common during ?

A

first thing in AM and drop in blood pH overnight - improves throughout the day
episodic hemoglobinuria*

83
Q

the diagnostic gold standard for PNH is…

A

flow cytometry

84
Q

TX for severe or aplastic anemia from PNH

A

allogeneic hematopoetic stem cell transplant

85
Q

TX for severe hemolysis or thrombosis from PNH

A

eculizumab

86
Q

what supportive medication may reduce hemolysis for PNH

A

corticosteroids

87
Q

acute anemia due to blood loss is commonly caused by

A

direct RBC loss
- posthemorrhagic anemia
- external - trauma, postpartum hemorrhage
- internal - GI bleed, ruptured ectopic pregnancy, ruptured spleen, subarachnoid hemorrhage

88
Q

chronic anemia due to blood loss is commonly caused by

A

depletion of iron stores = iron deficiency anemia

89
Q

process of anemia due to blood loss

A
  1. hypovolemia - risk of organ hypoperfusion, CBC does not show anemia*
  2. anemia - compensatory mechanisms = hypovolemia resolves = CBC now shows anemia
  3. recovery - marrow replaces lost RBC with new, transient reticulocytosis
90
Q

compensatory mechanisms during anemia due to blood loss

A

vasoconstriction
fluid retention
shift of fluid from extravascular to intravascular
IV fluids

Hem/Onc (13 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions