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anemia pt 1 Flashcards

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1
Q

what makes someone anemic?

A

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

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2
Q

3 major causes of anemia

A
  1. decreased in RBC production
  2. increased destruction of RBC
  3. increased blood loss
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3
Q

2 classifications of hemolytic anemia

A
  1. intravascular - RBCs lyse within BLOOD VESSELS
  2. extravascular - RBCs are destroyed within ORGANS
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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

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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

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6
Q

for extravascular hemolytic anemia, RBC is destroyed in the ?

A

spleen (mostly)
liver

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7
Q

PE findings for hemolytic anemia

A

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

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8
Q

lab findings for hemolytic anemias

A

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

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9
Q

what can falsely elevate haptoglobin lvls

A

inflammatory markers

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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)
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11
Q

cause of hereditary spherocytosis

A

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

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12
Q

pathology of hereditary spherocytosis

A

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

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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

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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
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15
Q

3 types of hemoglobin

A
  1. hbA - 97-99% of Hb - “adult” hgb
  2. hbA2 - 1-3%
  3. hbF - <1%
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16
Q

chromosome 16 has 2 copies of ?

A

alpha-globulin gene - 4 total

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17
Q

chromosome 11 has 1 copy of ?

A

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

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18
Q

cause of alpha thalassemias

A

gene deletion = reduced alpha-chain synthesis

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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
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20
Q

demographics of alpha thalassemias

A

SE asians and chinese descent
mediterranean or african descent

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21
Q

Hb electrophoresis of alpha thalassemias would present ?

A

normal - equal proportion of hbA, hbA2, hbF

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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

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23
Q

how would the electrophoresis present in alpha thalassemia

A

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

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24
Q

3 normal alpha-globulin genes result in ?

A

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

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25
2 normal alpha globulin genes result in ?
alpha thalassemia MINOR HCT - 28-40% MCV - 60-75%
26
1 normal alpha globulin gene results in ?
hemoglobin H disease (HbH) has 4 beta-chains = little to no use for O2 transport HCT - 22-32% MCV - 60-70%
27
no normal alpha globulin genes result in ?
hydrops fetalis die in utero
28
describe the peripheral smear findings of alpha thalassemia trait
1. hypochromatic, microcytic cells 2. target cells
29
describe the peripheral smear findings of HbH
1. hypochromatic, microcytic cells 2. target cells 3. poikilocytosis 4. sometimes some normal RBC bc of transfusions
30
treatment for alpha thalassemia
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
31
cause of beta thalassemias
gene point mutation = reduced beta-chain synthesis
32
types of betathalassemias
1. beta+ - reduced beta-chain synthesis 2. beta0 - absent beta-chain synthesis
33
how would the electrophoresis of beta thalassemias present?
increased proportion of HbA2 and HbF (HbF > HbA2)
34
demographics of beta thalassemias
mainly mediterranean descent can be seen in african and asian descent
35
pathology of beta thalassemias
- 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)
36
lab findings of beta thalassemia
hgb/hct - decreased RBC - increased MCV - decreased reticulocyte - increased MCH - decreased
37
exam findings of beta thalassemia
"chipmunk facies" thinning of long bones pathologic fracture failture to thrive
38
describe the peripheral smear findings of beta thalassemia minor
hypochromic, microcytic cells target cells
39
describe the peripheral smear findings of beta thalassemia intermedia
hypochromic, microcytic cells target cells poikilocytosis
40
describe the peripheral smear findings of beta thalassemia major
hypochromic, microcytic cells target cells poikilocytosis nucleated RBC
41
treatment for beta thalassemia including definitive tx
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
sickle cell disease is an ____ ____ inherited disease
autosomal recessive does NOT affect a sex more than the other*
43
cause of sickle cell disease
abn substitution of an amino acid in the beta chain (betaS)
44
for sickle cell, if you are heterozygous it would be ____, while homozygous would be _____
sickle cell trait sickle cell anemia
45
demographics of sickle cell disease
African americans 1 in 400 black children also mediterranean, latin, and native american descent
46
what % of African Americans make up carriers in the US
8%
47
pathology of sickle cell disease
unstable HbS polymerizes = sickled shaped = vaso-occlusive episodes "stick" to endothelium vasoocclusion = ischemia, pain, end-organ damage
48
what sequesters and destroys sickled RBCs?
spleen
49
what triggers sickle cell disease?
anything stressful! hypoxemia, acidosis, infection, excessive exercise, abrupt temp changes, anxiety/stress
50
a pt with sickle cell disease would show what in their hemoglobin electrophoresis?
abnormal proportions of HbA, HbA2, HbF presence of HbS band
51
Howell-Jolly inclusion bodies would be seen in ___
sickle cell anemia
52
clinical presentations for sickle cell trait
often asymptomatic can have s/s during physical stress - high altitude, heavy exercise, dehydration
53
when do s/s for sickle cell disease appear?
around 6 months old
54
clinical presentations for sickle cell anemia
- poorly healing LE ulcers (10+ y/o) - dactylitis - vision changes, retina problems - splenomegaly (<3 y/o) - hyperdynamic precordium
55
clinical presentation of sickle cell crisis
- 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
how do you treat sickle cell anemia/what are the ways
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
what is the mainstay medication of sickle cell anemia treatment? what SE must you address
hydrourea bone marrow suppression - can cause skin ulcers - increase cancer risk -teratogenic
58
an acute sickle cell crisis treatment is...
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
definitive tx for sickle cell anemia
stem cell transplant
60
main cause of G6PD deficiency is...
enzyme deficiency x-linked recessive!!
61
G6PD deficiency is commonly seen in...
African american MALES (X-linked recessive) also in Asian and mediterranean descent
62
what anemia is seen when RBCs are vulnerable to oxidative stress and forms Heinz bodies
G6PD deficiency
63
Splenomegaly is usually not seen in G6PD deficient pts because...
it is an episodic hemolytic anemia
64
what are the 3 major triggers of G6PD deficiency
1. infections 2. foods - FAVA BEANS 3. medications - ABX (sulfas, quinolones, nitrofurantoin), Azo, aspirin
65
dark urine can be seen in G6PD deficiency because ...
it is INTRAVASCULAR
66
a peripheral smear with bite cells, blister cells, and Heinz bodies is seen in...
G6PD deficiency
67
avoiding oxidant drugs is a preventive measure for what anemia
G6PD deficiency
68
cause of autoimmune hemolytic anemia
1. antibodies form against RBC antigens (igG) - 50% idiopathic - diseases - SLE, leukemia, lymphoma
69
autoimmune hemolytic anemia must be distinguished from...
drug-induced immune hemolytic anemia - caused by abx (cephalosporins, PCNs, quinolones), methyldopa or levodopa, or NSAIDs
70
pathology of autoimmune hemolytic anemia
RBC tagged for destruction spherocytes formation
71
what anemia can have complements tag RBC for destruction by Kupffer cells
autoimmune hemolytic anemia
72
how does IgM play a role in autoimmune hemolytic anemia
facilitates MAC-induced direct intravascular hemolysis
73
what type of hemolytic anemia can be triggered from warm or cold temperatures
autoimmune hemolytic anemia
74
difference between direct and indirect Coombs test
direct = RBC - reagent mixed with pt RBC indirect = serum - pt serum is mixed with RBCs; reagent is added
75
marked microspherocytosis can be seen in ?
autoimmune hemolytic anemia
76
immunosuppression, comorbidities, and transfusions can be treatments for..
autoimmune hemolytic anemia severe = splenectomy or transfusion
77
cause of hemolytic disease of newborns
maternal IgG to antigens on surface of fetal RBC - fetal-maternal hemorrhage - hydrops fetalis
78
positive direct Coombs test can be seen on who during hemolytic disease of the newborn
newborn indirect = maternal
79
what is the preventive care for hemolytic disease of newborn
RhoGAM - prevents immune response to Rh+ blood in Rh- mother
80
an acquired genetic defect that leads to RBC lysis by complement
paroxysmal nocturnal hemoglobinuria
81
Free Hb from NH results in...
depleted NO esophageal spasms, ED, renal damage, thrombosis
82
PNH is common during ?
first thing in AM and drop in blood pH overnight - improves throughout the day episodic hemoglobinuria*
83
the diagnostic gold standard for PNH is...
flow cytometry
84
TX for severe or aplastic anemia from PNH
allogeneic hematopoetic stem cell transplant
85
TX for severe hemolysis or thrombosis from PNH
eculizumab
86
what supportive medication may reduce hemolysis for PNH
corticosteroids
87
acute anemia due to blood loss is commonly caused by
direct RBC loss - posthemorrhagic anemia - external - trauma, postpartum hemorrhage - internal - GI bleed, ruptured ectopic pregnancy, ruptured spleen, subarachnoid hemorrhage
88
chronic anemia due to blood loss is commonly caused by
depletion of iron stores = iron deficiency anemia
89
process of anemia due to blood loss
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
compensatory mechanisms during anemia due to blood loss
vasoconstriction fluid retention shift of fluid from extravascular to intravascular IV fluids

Hem/Onc (13 decks)

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