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Interesting case CT 50 YO Jaundice . Dr.Whitecar BMHGT 1/28/09. Eosinophilia (AEC>600). 3 CYTOKINE PATHWAYS FOR TRIGGER(production and longevity) IL5; IL3 AND GM-CSF Chemokines for migration Primary and secondary Clonal and idiopathic (HES) CEL chronic eosinophilic leukemia.
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Interesting caseCT 50 YO Jaundice Dr.Whitecar BMHGT 1/28/09
Eosinophilia (AEC>600) • 3 CYTOKINE PATHWAYS FOR TRIGGER(production and longevity) • IL5; IL3 AND GM-CSF • Chemokines for migration • Primary and secondary • Clonal and idiopathic (HES) • CEL chronic eosinophilic leukemia
Secondary Causes • Atopy –asthma • Diet and medication allergy • Helmenthic and fungal conditions • Drug reactions • Lymhomnas ; gastric/lung Ca(para); HTLV1; ATLL • Addison ‘s disease • RA;Toxic oil; CS vasculitis; trypto myalgia
CEL –chronic eosinophilic leukemia • Cytogenetic or molecular evidence of Clonal nature • Peripheral Blasts >2% • Bone marrow blasts >5% <19% • Other causes excluded • Ch-4q12 deletion ; FIP1L1-PDGFRA • CH1C2; BCR-PDGFRA&b; FGFR1 MUTATIONS
Hyper-eosinophilic Syndromes ( HESs) • Older terminology-idiopathic;loeffler • HESs- Chusid criteria • AEC >1500 • >6 months of hypereosinophilia • Organ involvement • Absence of allergic or parasitic conditions
Organ involvement • Blood 100% • 58% cardiac • Skin 56% • Neuro;lung 50% • Spleen 43% • Liver 30% • Ocular 23%-50%
Cardio-pulmonary manifestations • Endomyocardial (subtle) • Thrombotic • Fibrotic---CHF • Same 3 stages seen in other diseases with increased Eosinophils ( parasitic ) • Cough; lung infiltrates and fibrosis
GI Manifestations • Chronic active hepatitis • Budd Chiari like picture • Colitis • Esophagitis gastritis • Gastro enteritis
Ocular and Neurologic • Blurry vision • Choroidial abnormalities ( upto 50%) • Primary CNS or peripheral NS • Neuropathy • Thromboembolic phenomenon
Hematologic (100%) • Anemia • Low platelets • High platelets • Lymphadenopathy • Eosinophilia • Hypersplenism with painful enlarged spleen
Myeloproliferative variant • Affects males • FIP1L1-PDGFR ALPHA FUSION GENE MUTATION positive (+) • INCREASE B12; TRYPTASE • CARDIAC INVOLVEMENT • IMATINIB IS Rx
Lymphocytic variant • Male = females • FIP1L1-PDGFR ALPHA FUSION GENE MUTATION negative (-ive ) • Increase IGM;IgG ; • Thymus Chemokine (TARC) increased • Abnormal or clones of T Cells present • Good response to steroids