Chapter 13: Hematologic Indications of Liver Transplantation

HEMATOLOGIC DISORDERS

• Budd-Chiari syndrome (see chapter on Budd-Chiari syndrome)

• Portal vein thrombosis (see chapter on portal vein thrombosis)

• Bone marrow rejection (graft versus host disease)

• Congenital vascular malformations (hereditary hemorrhagic telangiectasia)

Hereditary Hemorrhagic Telangiectasia

• Autosomal dominant genetic etiology:

  • Gene mutations:

    • Endoglin (ENG)

    • Activin receptor–like kinase type 1 (ALK-1)

• Prevalence:

  • One to 2 cases/10,000

  • Only 75% of patients have vascular malformations in the liver demonstrable through sensitive imaging

  • Only 8% of patients with visible liver vascular malformations are symptomatic

• Pathophysiology (Fig. 13-1)

• Clinical features (Table 13-1)

  • The vascular malformations can involve the lung, brain, and/or liver (Fig. 13-2).

  • Liver symptoms appear around age 30

  • The 3 most common initial presentations are:

    • High-output heart failure

    • Portal hypertension

    • Biliary ischemia

• Diagnosis:

  • Angiography is the gold standard in the diagnosis of liver vascular malformations

  • Genetic studies: Two most common coding sequence mutations

    • ENG

    • ALK-1

  • Other alternatives are:

    • Doppler ultrasonography (showing enlarged hepatic artery and intrahepatic hypervascularization)

    • Multiphase computed tomography (CT) or magnetic resonance imaging (MRI), searching for heterogeneous hepatic enhancement (Fig. 13-3)

    • Magnetic resonance angiography (MRA) searching for the vascular malformation (Fig. 13-4)

• Management:

  • Symptomatic treatment:

    • Intensive medical therapy for high output heart failure

    • Cirrhosis management for portal hypertension

    • Biliary ischemia

      • Abdominal pain responds to nonsteroidal anti-inflammatory drugs (NSAIDs)

      • In case of cholangitis, add systemic antibiotics

      • Invasive biliary imaging procedures, such as endoscopic retrograde cholangiography, should be avoided

      • Ursodeoxycholic acid could be helpful

  • Shunt reduction treatment:

    • Antiangiogenic drugs such as bevacizumab could be helpful

    • Transarterial embolization

    • Surgical ligation of the hepatic artery

  • Liver transplant as final definitive curative option

Isolated Congenital Liver Shunts (Table 13-2)

HELLP Syndrome

• Risk factors:

  • Preeclampsia

    ...