autoimmune hemolytic anemia Flashcards

1
Q

immune hemolytic anemia

A

shortened RBC survival mediated through immune response typically humoral antibody

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2
Q

3 categories of hemolytic anemia

A

-alloimmune
-autoimmune
-drug induced (lumped with this because autocontrol and DAT are both positive)

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3
Q

most autoimmune antibodies react with what kind of antigens

A

high frequency

autoanti-I more likely

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4
Q

cold hemagglutinin

A

interfere with routine testing but otherwise unimportant

-become clinically sig as titer and reactive temp increases

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5
Q

who are cold hemagglutinin most common in

A

older patients tend to be chronic

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6
Q

cold agglutinins have reactivity at what temp

A

below 20

-don’t worry in routine testing

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7
Q

options for finding cold hemagglutinin

A

autoabsorption or prewarming

prewarming done for a screen

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8
Q

how to prepare a cold hemagglutinin warm incubations for ABO back typing

A

need to include a screening cell and read the tube without centrifugation

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9
Q

how to determine if cold auto relevant

A

pathological antibodies are reactive closer to body temp and have higher titer than benign ones

titer >1000 at 4 degrees to call relevant

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10
Q

most common kind of cold auto

A

anti-I
-confirm by testing patient plasma with cord cells which have almost no I antigen

autoanti-I= rare sequela of infectious mono

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11
Q

in cold hemagglutinin symptoms

A

extremities because colder and further away from flow

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12
Q

what does cold hemagglutinin look like in blood bank

A

screening cells positive
DAT positive
Auto 0-4+

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13
Q

what is paroxysmal cold hemoglobinuria

A

patient has a hemolytic event after exposure to cold temp

findings: intravascular hemolysis and hemoglobinuria

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14
Q

when is paraoxysmal cold hemoglobinuria seen

A

typically following viral infection : syphilis

rare

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15
Q

paroxysmal cold hemoglobinuria specifically caused by

A

biphasic antibody

bind RBC in cooler temp and when warm RBC lyse

usually IgG anti-P

Hgb can drop to 4-5

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16
Q

when paroxysmal cold hemoglobinuria ID it is called

A

Donath-Landsteiner antibody

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17
Q

PCH donath landsteiner test specimen

A

tests for biphasic antibody

serum from freshly coag blood - red tops; must have active complement

clotted and spun at 37 degrees to avoid auto absorption

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18
Q

donath landsteiner treatments

A

A tubes - 30 min ice bath, 60 min at 37

B tubes- 90 min ice bath

C tubes - 90 min at 37 degrees

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19
Q

3 sources of serum for donath landsteiner test

A

1- patient serum
2- normal serum and patient serum - controls for complement irregularities
3- normal serum

mix with normal RBCs ( have P antigen)

read hemolysis after 90 mini

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20
Q

normal person results for landsteiner test

A

no reactivity

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21
Q

landsteiner test results for PCH

A

Hemolysis in A test for patient serum (1) and patient and normal (2)

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22
Q

identifying characteristics of PCH

A

children/ young adults

hemolysis- intravascular

antibody class- IgG (anti-P)

donath landsteiner +

treatment= supportive

23
Q

identifying characteristics of Cold hem. disease

A

elderly patients

hemolysis- extravascular

antibody class- IgM (anti-I)

treatment= avoid cold

24
Q

how are most AIHA caused

A

warm antibody (70%)

both IgG and complement involved

(small minority caused by IgM, IgA)

25
onset of WAIHA
gradual, pallor, weakness, dizziness, jaundice, unexplained fever extravascular hemolysis
26
what does WAIHA look like
screening cells, DAT, and auto all positive
27
problem warm aiha present to blood banker
often need transfusions and their autoantibody obscures potentially sig alloantibodies
28
associated conditions to warm AIHA
leukemias, lymphomas, myelodysplastic disorders, autoimmune disorders, GI disorders, chronic renal failure
29
what subclass of IgG is severe for warm AIHA
odd number 3, 1 macrophages destroy RBC's with IgG and complement faster than just IgG
30
what is important in WAIHA
alloantibodies treated as more important than auto can show relative specificity- panreactivity but stronger reactions for cells with particular antigen
31
WAIHA testing will likely require
adsorption if patient recently transfused, autoabsorption is to be avoided= do differential absorption with R1R1, R2R2, rr cells
32
selecting units for WAIHA
match as close as possible, but if broad specificity may be need to issue least incompatible bc crossmatch will be incompatible -need pathologist and doctor to sign if alloantibody must give antigen negative blood do not give D + blood to D - patient to avoid autoantibody or others
33
treatment for WAIHA
corticosteroids (immunosuppressants) if that doesn't work- immunoglobulin treatment splenectomy considered if doesn't respond to high doses of steroids final= more harsh immunosuppressive drugs
34
why do WAIHA benefit from splenectomy
reduces amount of RES tissue and antibody production
35
drug induced hemolytic anemia
target in drug induced disorder, not normal RBC antigen on its own typically antibiotics are involved (penicillins)
36
treatment for drug induced hemolytic anemia
stop giving the drug -difficult decision if treatment is necessary drug administration is normally continued if DAT is positive with no evidence of hemolysis no clinically sig if only involved blood bank testing
37
hapten mechanism (drug absorption)
immune system forms an antibody response to the drug itself as it absorbs onto RBC's
38
how is hapten mechanism detected
drug coated screening cells -can be missed on normal antibody screen
39
what is a hapten
small molecule that has reactivity with an antibody -only reacts with large antibody (small molecule fixes to large molecule)
40
hapten mechanism looks like what on panel
dat + auto +
41
hapten mechanism looks like what on panel with drug coated cells
screening cells + DAT + Auto +
42
immune complex mechanism (innocent bystander)
drug complexes with serum proteins novel antigens activate an immune response igM binds drug complex and falls onto RBC's and activate complement (lyses nearby RBC)= intravascular hemolysis
43
how is immune complex mechanism different from hapton
immune complex- non-covalent associations of drug with RBC surface that produce neoantigen- becomes target of antibody
44
immune complex mechanism panel results
anti-C3./ poly DAT + auto +
45
immune complex mechanism panel results with drug added
screening cells positive anti-C3/poly + auto + elute -
46
membrane modification mechanism
drug modifies the RBC membrane which allows association/binding of many different plasma proteins (antibodies and complement) considered non immune because antibodies are totally non specific to RBC
47
what is membrane modification caused by (specific)
cephalosporins aggregate on RBC cause drug making it attractive
48
what does membrane modification mechanism look like on panel
anti-C3/poly/ and anti-IgG DAT + auto +
49
unifying hypothesis
comprehensive framework put forward to explain some messier realities of certain patients antibody targets parts of hapten/ RBC
50
antibody to drug
hapten (absorption) drug dependent (bystander)
51
antibody to drug and membrane components
hapten (absorption) drug dependent (bystander)
52
antibody mainly to membrane components
membrane modification mechanism
53
exception to unifying hypothesis
methyldopa treatment -known to induce production of an autoantibody against true RBC antigens drug induces
54
methyldopa panel reaction
DAT + autocontrol + eluate + SC +