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

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

Sep 15, 2022 230 likes •584 views

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 Patient background 17 y/o female Healthy No meds No

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Tom Konikoff 6.6.17

Internal medicine “D”

The curious case he curious case

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Eos Eosinophilia inophilia in in the the night night time time

ד תימינפ

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Patient background

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

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11/2016

Productive cough Fever Dyspnea

Another hospital

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11/2016

Another hospital

1900 17K 14 (N<5)

Leukocytosis Eosinophilia CRP

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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
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But 3 weeks later….

  • No longer on steroids
  • Throat pain, dyspnea
  • Eosinophil count  2400
  • Bilateral alveolar infiltrates
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Prednisone 30 mg

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Second episode

  • Eos count  9000
  • PLT  33K
  • Abdominal pain + light epigastric tenderness
  • Maculopapular rash
  • n limbs

and abdomen

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Left hepatic vein Right hepatic vein ?

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LHV MHV RHV

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12/2016

Due to susp. Budd-Chiari syn. transferred to Internal Medicine “D” Rabin Medical Center.

Eosiniophilic disease with

BUDD CHIARI SYNDROME

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in PNIMIT D

  • WBC 26K
  • EOS 6.1K
  • PLT 13K
  • CRP 6
  • Elevated liver enzymes

(AST 348, ALT 602)

  • INR (spont.) 1.57 ?
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v

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Portal vein Inferior Vena cava

Bypass (T.I.P .S)

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  • 1. Infectious
  • 2. Allergies and substances
  • 3. Idiopathic eosinophilic pneumonia
  • 4. Vasculitis
  • 5. Malignancies (Solid, Hematological)
  • 6. Hypereosinophilic syndrome

Budd-Chiari syndrome secondary to Hypereosinophilic syndrome

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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

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HES Primary (neoplastic) Secondary (reactive) Idiopathic

>80%

Up to 6.3 per 100,000

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

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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

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Factor I (Fibrinogen) MBP , EPO thrombomodulin Factor III (Tissue factor)

Platelets

Akuthota P et al. Eosinophils and Disease Pathogenesis. Semin

  • Hematol. 2012 Apr; 49 (2).
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But what about the treatment?

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Is an acute well-defined clot present? Contraindication for thrombolytic therapy? Symptomatic? Thrombolytic therapy successful?

No No No Yes Yes Yes

Angiography/ stenting successful? TIPS Continue anticoagulation

No No Yes Yes

Approach to Budd-Chiari in non-Cirrhotic patients

DeLeve et al. Vascular disorders of the

  • liver. Hepatology. 2009 May;49(5):1729-

64

AASLD practice Guidelines

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Is an acute well-defined clot present? Contraindication for thrombolytic therapy? Symptomatic? Thrombolytic therapy successful?

No No No Yes Yes Yes

Angiography/ stenting successful? TIPS Continue anticoagulation

No No Yes Yes

Approach to Budd-Chiari in non-Cirrhotic patients

DeLeve et al. Vascular disorders of the

  • liver. Hepatology. 2009 May;49(5):1729-

64

AASLD practice Guidelines

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But when is it best to TIPS?

Primary intervention After Angioplasty Recurrent/ Chronic Asymptomatic BCS Cirrhosis

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2008 2016

“good long-term results” Non comparison High risk Patients

PrimaryTIPS over primary Angioplasty

“better patency and less mortality with primary TIPS “

Small size, retrospective  small paper

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Primary Angioplasty Vs. Primary TIPS

?

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Whatever you choose…always treat the underlying cause!

 Steroids  Hydrea (in our case)

Idiopathic Hypereosinophilic Syndrome

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In summary

 Young healthy female  Idiopathic Hypereosinophilic syndrome  Secondary Budd-Chiari  T.I.P

.S (+ steroids and hydrea)

 Doing well (normal LFT )

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  • 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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  • ד תימינפ
  • ןוכמהדבכ
  • יגולוטמהה ןוכמ