autoimmune hemolytic anemia Flashcards
theories of autoimmunity
- malfunctioning T cells
- cross reactivity
- molecular mimicry
- alteration of self antigens
- secondary to disease
- inherited tendency
primary idiopathic autoimmunity
no known cause
found in elderly
autoimmunity secondary to disease
condition associated with autoimmunity
- CLL leukemia, infections and viral infections (EBV), mycoplasma pneumoniae
drug related hemolysis
certain drugs that can cause hemolysis
seen as an interference
most common type of AIHA
warm autoimmune hemolytic anemia
other types of AIHA
cold autoimmune hemolytic anemia
drug related hemolytic anemia
what can cause a positive DAT
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
warm AIHA
primary idiopathic
secondary:
- white cell malignancies
- autoimmune diseases (lupus, rheumatoid arthritis)
- viral infections
WAIHA symptoms
- pallor
- weakness
- shortness fo breath
- dizziness
- jaundice
- fever
- splenomegaly
hematology results of a WAIHA
Hb and Hct decreased
periph smear : nucleated rbc, polychromasia, spherocytes, howell jolly
other indicators of extravascular hemolysis
serological findings of a WAIHA
ABO Rh norm
Ab screen pos
Dat pos
Ab ID panel panreacting (all the same grading)
Xm incompatible
antibody ID of a WAIHA
rules outs need negative, impossible to ID in WAIHA
broad specificity in Rh system
specificity sometime demonstrated
follow up to a WAIHA
- 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
first line of WAIHA treament
corticosteroids
- immunosuppresion will slow down the removal of red cells
- macrophages will be more suppressed
second line of WAIHA treatment
splenectomy - reduces removal
rituxamab (anti-CD20) - will get rid of B cells
third line of WAIHA treatment
cyclophosphamide / azathiaprine
- heavy duty chemo