Eosinophilia Flashcards
Initial treatment of hyperoeosinophilia patients that are sick
High dose steroids
Gene rearrangements in myeloid/lymphoid neoplasms associated with eosinophilia
- PDGFRA, PDGFRB,
- FGFR1, PCM1-JAK2,
- FLT3
- ETV6-ABL1 fusion
Chronic eosinophilic leukemia clinical features
Persistent eosinophilia >1.5 x 10^9/L
No evidence of disease defining fusion proteins or molecular markers (ie- BCR-ABL, PDGFR etc) or >10% of WBCs
Abnormal BM morphology
Presence of a clonal marker (cytogenetic or molecular) and if no mutation, other etiologies excluded
>2% PB blasts or >5% marrow blasts but <20%
peak incidence 4th decade
Symptomatic - fever, fatigue, cough, pruritus, diarrhea, angioedema, myalgia, and end-organ damage
General goal of treatment in CEl and HUS
Goal is to decrease eosinophil count to mitigate organ damage and prevent thrombosis
Initial treatment of HES
prednisone 1 mg/kg
taper slowly to lowest effective dose
IF remission, maintain on prednisone 10 mg or lower indefinitely
Other therapies for HES
Hydroxyurea as steroid sparing agent
Anti-IL-5 therapy (mepolizumab), anti-IL-5 receptor (benralizumab)
IFNalpha (also an option)
Mepolizumab mechanism
Anti-IL5
benralizumab mechanism
Anti-IL5
IF hypereosinophilic condition associated with PDGFRA, what is the treatment?
Imatinib
Significance of FGFR1 with myeloid neoplasms with hypereosinophilia
- aggressive, high rate of transformation to AML or T-ALL
Pemigatinib SE”s
- hyperphosphatemia
- alopecia
- diarrhea
- stomatitis
- anemia