Aplastic anemia Flashcards
What is ATG?
horse or rabbit-derived antibodies against human T cells and their precursors (thymocytes)
promacta generic name
eltrombopag
Pathophysiology of aplastic anemia
immune injury to multipotent hematopoietic stem cells
Etiologies of aplastic anemia
1) Autoimmune (most cases) –> thus idiopathic…
2) Direct injury (drugs, chemicals, radiation)
3) Viral infection
4) clonal and genetic disorders
* shared though, drugs/chemicals/viruses are thought to alter the appearance of HSCs and lead to immune destruction
BMB result with aplastic anemia
profoundly hypocellular with a decrease in all elements; the marrow space is composed mostly of fat cells and marrow stroma
Treatment of severe aplastic anemia
IF candidate + younger than 40 –> allogeneic HCT
IF over age 40 –> horse ATG, cyclosporine, and eltrombopag
IST stands for
immunosuppressive therapy
Management of moderate aplastic anemia
Lower intensity immunosuppressive therapy or single-agent EPAG
Horse/equine ATG vs. rabbit ATG in terms of efficacy
Equine ATG has proven to be more effective in reducing remission (rabbit is second line)
Biggest SE with ATG
Infusion reactions, anaphylaxis, serum sickness
Why steroids are used with IST
reduce incidence of serum sickness
Second line if failed first line
If transplant candidate –> haplo transplant
If not transplant candidate –> 2nd IST + TPO mimemetic (eltrombopag)
sequelae of AA
70% go on to develop clonal hematopoesis
BMB in aplastic anemia findings
- severe hypocellularity
- no blasts
- no multilineage dysplasia
- no marrow fibrosis
Other item primarily on differential to differentiate AA from after BMB
hypocellular MDS
