The curious case he curious case of of Eos Eosinophilia - PowerPoint PPT Presentation

The curious case he curious case of of Eos Eosinophilia inophilia in in the the night night time time Tom Konikoff 6.6.17 Internal medicine “D” ד תימינפ

Patient background 17 y/o female Healthy No meds No drugs/ doesn't smoke No known allergies No relevant personal of family medical history

11/2016 Fever Productive cough Dyspnea Another hospital

11/2016 Leukocytosis 17K Eosinophilia 1900 CRP 14 (N<5) Another hospital

First episode  Blood, Urine, sputum, fecal cultures - NEG  Serology for EBV , CMV , Q - FEVER - NEG  Eosinophilic pneumonia  Prednisone  Clinical improvement, fever , Eosinophil count , CXR improves  Discharge with steroid tapering down for 3 weeks

But 3 weeks later….  No longer on steroids  Throat pain, dyspnea  Eosinophil count  2400  Bilateral alveolar infiltrates

Prednisone 30 mg

Second episode  Eos count  9000  PLT  33K  Abdominal pain + light epigastric tenderness  Maculopapular rash on limbs and abdomen

Right hepatic vein ? Left hepatic vein

LHV MHV RHV

12/2016 Due to susp. Budd-Chiari syn. transferred to Internal Medicine “D” Rabin Medical Center. Eosiniophilic disease with BUDD CHIARI SYNDROME

in PNIMIT D  WBC 26K  INR (spont.) 1.57 ?  EOS 6.1K  PLT 13K  CRP 6  Elevated liver enzymes (AST 348, ALT 602)

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Inferior Vena cava Bypass (T.I.P .S) Portal vein

1. Infectious Budd-Chiari syndrome 2. Allergies and substances secondary to 3. Idiopathic eosinophilic pneumonia 4. Vasculitis Hypereosinophilic 5. Malignancies (Solid, Hematological) syndrome 6. Hypereosinophilic syndrome

Hematological Diseases & Budd-Chiari syn. Hypercoagulable state (JAK2, factor V Leiden, Erythrocytosis) • 49% of Budd-Chiari cases are due to myeloproliferative • disorders May be presenting symptom • Patients with Splanchnic vein thrombosis (including budd-chiari) • and no underlying disorder identified  JAK2 testing Smalber et al. Myeloproliferative neoplasms in Budd-Chiari syndrome and portal vein thrombosis: a meta-analysis . Blood. 2012 Dec;120(25):4921-8

Up to 6.3 per 100,000 HES Primary Secondary Idiopathic (neoplastic) (reactive) >80% Crane et al. Incidence of myeloproliferative hypereosinophilic sy ndrome in the United States and an estimate of all hypereosinophilic syndrome incidence. J Allergy Clin Immunol. 2010 Jul;126(1):179-81

Eosinophilia & Hypercoagulability (Budd-Chiari syndrome) • Akuthota P et al. Eosinophils and disease pathogenesis. Semin Hematol. 2012 Apr; 49 (2) • Sharma SK et al . Eosinophilia: Rare cause of arterial thrombosis and cardioembolic stroke in childhood . World J Cardiol. 2012 Apr 26;4 (4):128-9  Vazques et al. Coagulation abnormalities in patients with eosinophilia . Postgrad Med J. 1987 Nov; 63

Factor III (Tissue factor) Factor I (Fibrinogen) MBP , EPO thrombomodulin Platelets Akuthota P et al. Eosinophils and Disease Pathogenesis . Semin Hematol. 2012 Apr; 49 (2).

But what about the treatment?

DeLeve et al . Vascular disorders of the Approach to liver. Hepatology. 2009 May;49(5):1729- Is an acute well-defined 64 Budd-Chiari clot present? Yes in non-Cirrhotic No Contraindication for patients AASLD practice thrombolytic therapy? Guidelines No Yes Symptomatic? Thrombolytic therapy Yes successful? No No Yes Angiography/ stenting successful? Continue TIPS anticoagulation No Yes

DeLeve et al . Vascular disorders of the Approach to liver. Hepatology. 2009 May;49(5):1729- Is an acute well-defined 64 Budd-Chiari clot present? Yes in non-Cirrhotic No Contraindication for patients AASLD practice thrombolytic therapy? Guidelines No Yes Symptomatic? Thrombolytic therapy Yes successful? No No Yes Angiography/ stenting successful? Continue TIPS anticoagulation No Yes

But when is it best to TIPS? Primary intervention After Angioplasty Recurrent/ Chronic Asymptomatic BCS Cirrhosis

2016 2008 “good long - term results” Non comparison High risk Patients Primary TIPS over primary Angioplasty “better patency and less mortality with primary TIPS “ Small size, retrospective  small paper

Primary Angioplasty Vs. Primary TIPS ?

Whatever you choose…always treat the underlying cause!  Steroids (in our case)  Hydrea Idiopathic Hypereosinophilic Syndrome

In summary  Young healthy female  Idiopathic Hypereosinophilic syndrome  Secondary Budd-Chiari  T.I.P .S (+ steroids and hydrea)  Doing well (normal LFT )

Budd-Chiari syn. may be the presenting symptom of • many hematological disorders – not only Myeloproliferative Eosinophilia is a precipitating factor for • splanchnic thrombotic events Early TIPS may be considered •

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