autoimmune hemolytic anemia Flashcards

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1
Q

theories of autoimmunity

A
  • malfunctioning T cells
  • cross reactivity
  • molecular mimicry
  • alteration of self antigens
  • secondary to disease
  • inherited tendency
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2
Q

primary idiopathic autoimmunity

A

no known cause
found in elderly

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3
Q

autoimmunity secondary to disease

A

condition associated with autoimmunity
- CLL leukemia, infections and viral infections (EBV), mycoplasma pneumoniae

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4
Q

drug related hemolysis

A

certain drugs that can cause hemolysis
seen as an interference

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5
Q

most common type of AIHA

A

warm autoimmune hemolytic anemia

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6
Q

other types of AIHA

A

cold autoimmune hemolytic anemia
drug related hemolytic anemia

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7
Q

what can cause a positive DAT

A

in vivo binding of immunoglobulin or complement or both
- patient alloantibodies bind to donor cells
- maternal antibodies bind to fetal cells
- passive antibodies
- autoantibodies
- antibodies/complement due to drugs

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8
Q

warm AIHA

A

primary idiopathic
secondary:
- white cell malignancies
- autoimmune diseases (lupus, rheumatoid arthritis)
- viral infections

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9
Q

WAIHA symptoms

A
  • pallor
  • weakness
  • shortness fo breath
  • dizziness
  • jaundice
  • fever
  • splenomegaly
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10
Q

hematology results of a WAIHA

A

Hb and Hct decreased
periph smear : nucleated rbc, polychromasia, spherocytes, howell jolly
other indicators of extravascular hemolysis

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11
Q

serological findings of a WAIHA

A

ABO Rh norm
Ab screen pos
Dat pos
Ab ID panel panreacting (all the same grading)
Xm incompatible

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12
Q

antibody ID of a WAIHA

A

rules outs need negative, impossible to ID in WAIHA
broad specificity in Rh system
specificity sometime demonstrated

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13
Q

follow up to a WAIHA

A
  • elution : acid elution woul dbe pos -> Ab bound to patient cells
  • crossmatch
  • transfusion (last option): Rh phenotype would have to be matched
    can use warm autoadsorption to ID alloantibodies
    transfuse lease incompatible
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14
Q

first line of WAIHA treament

A

corticosteroids
- immunosuppresion will slow down the removal of red cells
- macrophages will be more suppressed

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15
Q

second line of WAIHA treatment

A

splenectomy - reduces removal
rituxamab (anti-CD20) - will get rid of B cells

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16
Q

third line of WAIHA treatment

A

cyclophosphamide / azathiaprine
- heavy duty chemo

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17
Q

WAIHA procedure

A
  1. autoantibody is removes from patient cells by pre warming and washing. ZZAP used to remove IgM autoantibodies
  2. aliquot of patient plasma is incubated with dissociated patient cells at 4 degrees followed by centrifugation and removal of the auto adsorbed plasma
  3. the autoadsorbed plasma can be used for pre transfusion testing but prewarming may still be needed if autoantibody remains
18
Q

immunoglobulin class of a cold autoantibody

A

Igm

19
Q

thermal range of cold autoantibody

A

4, 15, 20, 35 degrees

20
Q

T or F: cold autoantibody can bind complement

A

T

21
Q

what is the specific antibody of cold

A

Anti-I Anti-i

22
Q

harmless classification of auto anti I

A

titre <64
max thermal range is 20-24
rxn not readily enhanced by albumin
polyclonal

23
Q

pathological classfification of auto anti I

A

titre >1000
max thermal range 30-32
rxn enhanced by albumin
monoclonal

24
Q

cold antibody AIHAs

A

primary idiopathic
secondary to disease
- mycoplasma pneumoniae: anti-I
EBV: anti-i

25
Q

etiology of cold autoantibody

A

causes harm at a high titre and will react at 30-32 will react and bind to their own cells
- IgM binds to I antigen (which will attach complement)

26
Q

clinical symptoms of a cold antibody

A
  • fingers, toes, nose below 30 degrees
    -anemia symptoms after exposure
  • body parts will go blue due to hemolysis extravascularly, agglutination will cause low oxygen blood in the tissue.
27
Q

hematological findings of cold antibody

A

increased LD
decreased Hb
decreased Hct
decreased haptoglobin

28
Q

treatment of cold AIHA

A

stay warm

29
Q

what is the name of low oxygen blood in tissueq

A

acrocyanosis

30
Q

how is autoantibody ID’d in a CHD

A

panel: IS
autocontrol: pos
cord cells: neg
titration: see if titre is changing

31
Q

what is the results of a DAT in CHD

A

polyspecific -> pos
monospecific -> C3d pos, IgG neg

32
Q

what is the procedure of a cold autoantibody titration

A
  1. prewarms patient plasma is diluted
  2. tested with adult O cells, patient cells, and cord cells at various temperatures
  3. titre determine at 4 degrees
  4. used strength of adult vs cord cells to determine identity
33
Q

what is the resolution of an anomalous result of ABO Rh testig

A

would do a cold autoadsorption and resolved by pre warming everything

34
Q

how do you find compatible blood in a CHD investigation

A
  • prewarm
  • use autoadsorbed plasma
  • ensure monospecific anti-IgG used
  • ensure EDTA sample used
  • transfuse using warmer blood if necessary
35
Q

what will happen is poly specific used in a CHD

A

false positive

36
Q

what is paroxysmal cold hemoglobinuria

A
  • anti-P : Donath-Landsteiner antibody
  • IgG
  • can bind complement
  • hemolyisis
37
Q

what is the etiology of a PCH

A

anti-P will react in rhe cold which fixes complement, affecting the MAC causing intravascular hemolysis which results in hemoglobinuria

38
Q

clinical symptoms of PCH

A

liver disease
temporary kidney disease

39
Q

lab findings of a PCH

A

decreased red cell count
increased LD
pos plasma hemoglobin
BM trying to compensate: will find NRBC, poly,
increase bilirubin
haptoglobin undetectable

40
Q

what is the procedure of the donath landsteiner test

A
  1. collect blood, keep warm, allow to clot
  2. incubate serum with P po rbc and noral serum at 4 degrees
  3. warm to 37 degreed
  4. spin and read for hemolysis

hemolysis = anti-P present