One Flashcards
Def of sAA
2 of 3 of the following: ANC sub 500, Plt sub 20k, relic <1 corrected or absolute less than 40000/microL AND 1 of 2 marrow criteria (<25% cellularity or cellularity 25-50 with <30% hematopoietic cells). Very severe is ANC <200
four nutritional def ass with sAA
zinc, copper, B12, folate
name two primary immune diseases associated with sAA
eosinophilic fasciitis, hypogamma
name 3 disorders associated with sAA
thymoma, large granular lymphocytic leukemia (adults usually), PNH, MDS
two increased lab findings seen in acquired AA
fetal hgb and āIā antigen increased
timing for hepatitis associated aplastic anemia
can happen after hepatitis resolves, happens in 30% of patients who get a transplant
what are the tests to rule out inherited BMF syndromes
chromosome breakage with diepoxybutane (DEB) or mitomycin C (MMC) and telomere length
which type of ATG is better
ATG is horse and is better. ALG is rabbit and is worse
IST treatment for SAA
ATG more than 150mg/kg (40 mg/kg/day x 4 d), steroids for 10-28 days to prevent serum sickness, and CYCLOSPORINE for 12 months with at least 3 months of stable counts before taper
timeline of serum sickness after ATG
5-10 days after starting
how does eltrombopeg work
non peptide thrombopoietin receptor antagonist. triggers receptor which lets to megakaryocytic differentiation
who needs eltrombopag dose reduction
southeast asian ancestry
toxicities and side effects- eltrombopag
monitor ALT/bili for liver stuff. skin rash, hyperpigmentation, cataracts
most patients with sAA respond by when
3-6 months
genetic aberration for PNH
acquired somatic mutations in PIG-A gene (Xp22.1) - without it, you have. deficiency in CD55/59 and get complement mediated lysis
risks of and complications of PNH
hemolysis, hemoglobinuria, and fatal thromboses (venous, mesenteric)
