autoimmune hemolytic anemia

Question 1

theories of autoimmunity

Answer 1

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

Question 2

primary idiopathic autoimmunity

Answer 2

no known cause
found in elderly

Question 3

autoimmunity secondary to disease

Answer 3

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

Question 4

drug related hemolysis

Answer 4

certain drugs that can cause hemolysis
seen as an interference

Question 5

most common type of AIHA

Answer 5

warm autoimmune hemolytic anemia

Question 6

other types of AIHA

Answer 6

cold autoimmune hemolytic anemia
drug related hemolytic anemia

Question 7

what can cause a positive DAT

Answer 7

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

Question 8

warm AIHA

Answer 8

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

Question 9

WAIHA symptoms

Answer 9

• pallor
• weakness
• shortness fo breath
• dizziness
• jaundice
• fever
• splenomegaly

Question 10

hematology results of a WAIHA

Answer 10

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

Question 11

serological findings of a WAIHA

Answer 11

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

Question 12

antibody ID of a WAIHA

Answer 12

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

Question 13

follow up to a WAIHA

Answer 13

• 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

Question 14

first line of WAIHA treament

Answer 14

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

Question 15

second line of WAIHA treatment

Answer 15

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

Question 16

third line of WAIHA treatment

Answer 16

cyclophosphamide / azathiaprine
- heavy duty chemo

Question 17

WAIHA procedure

Answer 17

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

Question 18

immunoglobulin class of a cold autoantibody

Answer 18

Igm

Question 19

thermal range of cold autoantibody

Answer 19

4, 15, 20, 35 degrees

Question 20

T or F: cold autoantibody can bind complement

Answer 20

T

Question 21

what is the specific antibody of cold

Answer 21

Anti-I Anti-i

Question 22

harmless classification of auto anti I

Answer 22

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

Question 23

pathological classfification of auto anti I

Answer 23

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

Question 24

cold antibody AIHAs

Answer 24

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

Question 25

etiology of cold autoantibody

Answer 25

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)

Question 26

clinical symptoms of a cold antibody

Answer 26

- 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.

Question 27

hematological findings of cold antibody

Answer 27

increased LD decreased Hb decreased Hct decreased haptoglobin

Question 28

treatment of cold AIHA

Answer 28

stay warm

Question 29

what is the name of low oxygen blood in tissueq

Answer 29

acrocyanosis

Question 30

how is autoantibody ID'd in a CHD

Answer 30

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

Question 31

what is the results of a DAT in CHD

Answer 31

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

Question 32

what is the procedure of a cold autoantibody titration

Answer 32

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

Question 33

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

Answer 33

would do a cold autoadsorption and resolved by pre warming everything

Question 34

how do you find compatible blood in a CHD investigation

Answer 34

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

Question 35

what will happen is poly specific used in a CHD

Answer 35

false positive

Question 36

what is paroxysmal cold hemoglobinuria

Answer 36

- anti-P : Donath-Landsteiner antibody - IgG - can bind complement - hemolyisis

Question 37

what is the etiology of a PCH

Answer 37

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

Question 38

clinical symptoms of PCH

Answer 38

liver disease temporary kidney disease

Question 39

lab findings of a PCH

Answer 39

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

Question 40

what is the procedure of the donath landsteiner test

Answer 40

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