idiopathic hypereosinophilia syndrome

Question 1

idiopathic hypereosinophilic syndrome:

Answer 1

proliferative disorder of eosinophils within the body, leading to organ damage (restrictive cardiomyopathy, valvular heart dx, pulmonary fibrosis and peripheral neuropathy)

Question 2

diagnosis of idiopathic hypereosinophilia syndrome:

Answer 2

see organ involvement

see peripheral eosinophilia >1500 cells/uL on two more occasions not due to other causes

Question 3

CHINA for eosionphilia

Answer 3

collagen - vascular disease (eosinophilic granulomatous with polyangiitis)
helminthic parasitic worm infection (strongyloides)
idiopathic hypereosinophilic syndrome (cause unknown after extensive evaluation)
Neoplasia - lymphomas most common myeloproliferative neoplasms
allergy - atopy asthma (drug induced like carbamazepines and sulfonamides)

Question 4

drug causes for peripheral eosinophilia

Answer 4

carbamazepine

sulfonamides

Question 5

eosinophilia is defined as:

Answer 5

sustained eosinophil counts >1500 and associated with end organ damage which affects the skin, lungs, heart, GI and brain

Question 6

what can also cause eosinophilia

Answer 6

Addison’s dx, Hodgkin lympohma
Job syndrome
cholesterol emboli
myeloproliferative variant of HEWS

Question 7

subtypes of idiopathic hypereosinophilia syndrome:

Answer 7

myeloproliferative varient of HES organomegaly and abnormalities of cell types

gene mutations

Question 8

management of idiopathic hypereosinophilia syndrome:

non gene mutated will have

Answer 8

close surveillance for end organ damage (heart and lungs)

some may need hydroxyurea and steroids.

Question 9

HES with FIP1L1 PDGRA mutation should receive this treatment

Answer 9

imatinib mesylate or Gleevec.

Question 10

differential diagnosis for eosinophilia on bronchoalveolar lavage

Answer 10

fungal infection
parasitic infection
drugs/toxins
lung irradiation
eosinophilic granulomatosis with polyangiitis
acute eosinophilic pneumonia

Question 11

to rule out incidental eosinophilia need to check:

Answer 11

history regarding exposures (travel, medications, illicit drug use, GI symptoms)
ANCA
coccidioides enzyme linked immunosorbent assay (ELISA)
Strongyloides ELISA
microscopic work up with sputum cultures and blood cultures.

Question 12

Hypereosinophilic syndrome is a form of

Answer 12

myeloproliferative disorder