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BSG Postgraduate Course Birmingham 2006. Haematological and vascular complications affecting the liver. Dominique-Charles Valla Hôpital Beaujon, Clichy, France. Blood disorders affecting the liver. Lymphoproliferative or myeloproliferative diseases Activated Macrophages

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haematological and vascular complications affecting the liver

BSG Postgraduate Course

Birmingham 2006

Haematological and vascular complications affecting the liver

Dominique-Charles Valla

Hôpital Beaujon, Clichy, France

blood disorders affecting the liver
Blood disorders affecting the liver
  • Lymphoproliferative or myeloproliferative diseases
  • Activated Macrophages
  • Lymphoproliferative diseases
  • Prothombotic disorders

Infiltration

Infiltration

Cytokine release

Light chain deposition

Thrombosis

prothrombotic disorders involvement of hepatic vessels
Prothrombotic DisordersInvolvement of hepatic vessels
  • Portal venous thrombosis

large- or small-sized veins

  • Hepatic venous thrombosis

large- or small-sized veins

  • Any combination thereof
secondary architectural changes
Secondary architectural changes

Vascular obstruction

Atrophy

Hypertrophy

Sinusoidal dilatation

Fibrosis

  • portal
  • central
  • sinusoidal
  • central
  • random
  • macronodules
  • micronodules
prothrombotic disorders affecting hepatic vessels
Prothrombotic disorders affecting hepatic vessels

Portal hypertension or Abnormal liver tests

  • Extrahepatic portal vein thrombosis

Pylephlebitis andPortal cavernoma

  • Hepatic vein/IVC thrombosis

Budd-Chiari syndrome

  • Intrahepatic vascular obstruction

Hepatic veins or Portal veins

  • Non-cirrhotic architectural changes
prothrombotic disorders in bcs or pvt
Prothrombotic Disorders in BCS or PVT

BCS PVT

Myeloproliferative diseases %

Hereditary thrombophilias %

Antiphospholipid syndrome %

PNH %

60 30 35 35

15 15

5 0

Janssen, Blood 2000. Deltenre, Gut, 2001. Primignani, Hepatology 2005

slide7

Case history

  • Healthy male patient, 39 year-old.
  • Enlarged spleen (6 cm) at routine examination
  • AST/ALT Normal WBC 9 000/fL
  • GGT & ALP 1.8xULN Platelets 250 000/fL Prothrombin 72% RBC 4.2 106/fL
  • Factor V 70% Hematocrit 39%
  • No cause for chronic liver disease
  • CT / US : Portal cavernoma.
  • Grade III esophageal varices with red signs
  • Needle biopsy: Normal liver
slide8

WBC 9 000/fL

Platelets 250 000/fL

Hematocrit 39%

Prothrombin 72%

Factor V 70%

Antithrombin N > 75% 70%

Protein C N > 65% 55%

Protein S N > 65% 62%

Factor V Leiden Absent

Factor II mutation Present

APL Ab/LA Absent

slide9

How many causal factors

have been fully identified ?

slide10

WBC 9 000/fL

Platelets 250 000/fL

Hematocrit 39%

Prothrombin 72%

Factor V 70%

Antithrombin N > 75% 70%

Protein C N > 65% 55%

Protein S N > 65% 62%

Factor V Leiden Absent

Factor II mutation Present

APL Ab/LA Absent

slide11

1

  • F II gene mutation
  • X

2

3

How many causal factors

have been fully identified ?

slide12

WBCC 9 000/fL

Platelets 250 000/fL

Hematocrit 39%

Prothrombin 72%

Factor V 70%

Antithrombin N > 75% 70%

Protein C N > 65% 55%

Protein S N > 65% 62%

Factor V Leiden Absent

Factor II mutation Present

APL Ab/LA Absent

slide13

PVT - Coagulation inhibitors

Fisher. Gut 2000; 46:534

slide14

WBCC 9 000/fL

Platelets 250 000/fL

Hematocrit 39%

Prothrombin 72%

Factor V 70%

Antithrombin N > 75% 70%

Protein C N > 65% 55%

Protein S N > 65% 62%

Factor V Leiden Absent

Factor II mutation Present

APL Ab/LA Absent

combination of prothrombotic disorders
Combination of prothrombotic disorders

BCS

PVT

At least 2 disorders (%) 25-35% 10-20%

Myeloproliferative disease in 20-60%

of patients with hereditary thrombophilias

Denninger. Hepatology 2000. Janssen Blood 2000.

Primignani Hepatology 2005

slide16

WBCC 9 000/fL

Platelets 250 000/fL

Hematocrit 39%

Prothrombin 72%

Factor V 70%

Antithrombin N > 75% 70%

Protein C N > 65% 55%

Protein S N > 65% 62%

Factor V Leiden Absent

Factor II mutation Present

APL Ab/LA Absent

bcs or pvt features of myeloproliferative disease
BCS or PVTFeatures of Myeloproliferative Disease

PPV

Δ Spleen > 5 cm

Platelets > 200 000/fL

100%

Chait et al. Br J Haematol 2005

myeloproliferative diseases
Myeloproliferative diseases

Diagnostic criteria BCS PVT

  • Classical criteria (PVSG) % 10 0
  • Endogenous erythroid colonies % 60 30
  • Bone marrow biopsy % 60 30
  • V617F JAK2 mutation % 60 30

James Nature 2005. Kralovics NEJM 2005. Baxter Lancet 2005. Levine Cancer Cell 2005.

Patel RK et al. ASH Dec 2005. Fabris FH et al. EASL 2006

slide19

F II gene mutation

  • Myeloproliferative disease
  • Portal vein thrombosis
  • Large oesophageal varices with red signs
disease specific antithrombotic therapies

Disease-specific Antithrombotic Therapies

  • Myeloproliferative diseases
    • Hydroxyurea
    • Low dose aspirin
    • Anagrelide
  • Other acquired or inherited conditions
    • Little or no data

Data still unclear for venous thromboses

Cortelazzo NEJM 1995. Landolfi NEJM 2004. Eliott Br J Haematol 2004. Crother Thromb Res 2004. Harrisson NEJM 2005

slide22

Chronic Portal Vein Thrombosis

Anticoagulation

Anticoagulation

yes

yes

no

no

17

6.0

p = 0.212

per 100 patients

per year

p = 0.015

7

1.2

Bleeding

Thrombosis

Condat et al. Gastroenterology 2001; 120:490

slide23

Chronic PVT – GI Bleeding

Prophylaxis

no

yes

24

17

per 100 patients

per year

Moderate/large-sized

varices

Condat et al. Gastroenterology 2001;120:490-497

slide24

Chronic portomesenteric venous thrombosis

Hazard Ratio for Death

1.00

1.00

p=0.030

p=0.038

0.28

0.10

yes

no

no

yes

Beta-blockers

Warfarine

Orr et al. Hepatology 2005; 42: 212A (AASLD San Francisco 2005)

slide25

Acute Portal Vein Thrombosis

Recanalisation

83%

75 %

Thrombolysis

(in situ, n = 20)

Anticoagulation

(alone, n = 27)

Condat.

Hepatology 2000

Holliingshead.

J Vasc Interv Radiol 2005

slide26

Acute Portal Vein Thrombosis

100

Major Bleeding

%

60%

5%

0

Thrombolysis

(in situ, n = 20)

Anticoagulation

(alone, n = 27)

Condat.

Hepatology 2000

Holliingshead.

J Vasc Interv Radiol 2005

portal vein thrombosis current guidelines in beaujon

PortalVeinThrombosisCurrent guidelines in Beaujon

Permanent prothrombotic disorder

→ Permanent anticoagulation

No contraindication

Prophylaxis for PHT-related bleeding

anticoagulation for bcs

Anticoagulation for BCS

  • Anticoagulation recommended to all patients, in the absence of major contraindication.
  • Previous bleeding from portal hypertension is not considered a major contraindication, provided appropriate prophylaxis for recurrent bleeding is initiated.

Janssen et al, J Hepatol 2003. de Franchis, J Hepatol 2005.

conclusions

Conclusions

  • Blood disorders are major causes of vascular liver diseases.
  • Atypical myeloproliferative diseases most commonly implicated.
  • Frequent combination of several causes.
  • Permanent anticoagulation is generally recommended once prophylaxis for portal hypertensive bleeding has been instituted.