How is hypersplenism managed?

Management of Hypersplenism

The management of hypersplenism should prioritize targeted interventions based on etiology, with thrombopoietin receptor agonists being first-line for procedure-related thrombocytopenia and partial splenic embolization being the preferred interventional approach for long-term management in suitable candidates. 1

Understanding Hypersplenism

• Hypersplenism is a common condition in patients with advanced cirrhosis and portal hypertension, characterized by peripheral cytopenia (most commonly thrombocytopenia) despite normal bone marrow function 1
• The pathophysiology is multifactorial, involving:
  • Portal hypertension with splenic sequestration of blood cells
  • Myeloid toxicity
  • Anti-platelet antibodies
  • Low thrombopoietin levels 1
• It's important to note that low platelet counts alone don't necessarily predict bleeding risk in cirrhotic patients and should be evaluated within the broader context of cirrhosis-affected hemostasis 1

Diagnostic Approach

• Diagnosis involves comprehensive evaluation including:
  • Complete blood count showing cytopenia (one or multiple cell lines)
  • Imaging studies (CT scan, hepatic ultrasonography) to assess splenic size and portal system 1
  • Rotational thromboelastometry for more accurate bleeding risk assessment before procedures 1
  • In cases of splenic vein thrombosis, additional angiography and bone marrow biopsy may be needed 1
• JAK2V617F mutation testing is helpful in cases of Budd-Chiari syndrome and portal vein thrombosis 1

Management Strategies

Pharmacological Management

• Thrombopoietin receptor agonists (avatrombopag, lusutrombopag) are first-line therapy before high-risk procedures or in the presence of bleeding 1
  • These agents effectively increase platelet counts and are superior to no treatment in avoiding platelet transfusion and rescue therapy 1
• Platelet transfusions can be used synergistically with local hemostatic measures in patients requiring high-risk procedures or with active bleeding 1

Interventional Procedures

• Partial splenic embolization (PSE) is the preferred interventional approach for long-term management of hypersplenism 1, 2

  • PSE effectively reduces splenic volume and portal pressure 1
  • The procedure involves embolizing 60-70% of the spleen using microspheres delivered via the splenic artery 2
  • PSE can resolve hemorrhage without recurrence in patients with gastric varices and splenic vein occlusion 1
  • Follow-up studies show normalization of blood counts in most patients within 4-20 months 2
  • Common side effects include abdominal pain and subfebrility for approximately 2 weeks post-procedure 2

• Splenic vein recanalization, including transjugular recanalization with angioplasty or stenting, has shown resolution of upper GI bleeding without recurrence 1

Surgical Management

• Surgical options include:

  • Proximal splenorenal shunt - not only relieves hypersplenism but also addresses underlying portal hypertension complications 3
  • Splenectomy (open or laparoscopic) - most effective for normalizing blood counts but carries significant risks including portal vein thrombosis 4
  • Partial splenectomy - preserves some splenic function while reducing hypersplenism 5

• Surgery effectively normalizes hypersplenism in most patients, with shunt procedures providing additional benefit of managing portal hypertension 3

Special Considerations

• In liver transplant recipients, portal pressure decreases rapidly post-transplant, with platelet counts usually normalizing within 2 weeks 1
• Subclinical hypersplenism may persist in patients with pre-transplant splenomegaly 1
• Patients with severe hypersplenism affecting all three cell lines (red cells, white cells, platelets) typically have:
  • Higher portal pressures
  • Increased risk of variceal bleeding
  • Higher incidence of portal biliopathy and gastropathy
  • Greater intraoperative blood loss during surgical interventions 3

Treatment Selection Algorithm

1. For asymptomatic hypersplenism with mild cytopenia:

   • Observation is appropriate as hypersplenism alone has little clinical consequence 4

2. For patients requiring procedures with thrombocytopenia:

   • Thrombopoietin receptor agonists are first-line therapy 1
   • Platelet transfusions can be used if needed for urgent procedures 1

3. For symptomatic hypersplenism with recurrent bleeding, infections, or severe cytopenia:

   • Consider partial splenic embolization as first interventional approach 1, 2
   • If PSE fails or is contraindicated, consider surgical options based on underlying etiology:
     • For non-cirrhotic portal hypertension: proximal splenorenal shunt 3
     • For cases where shunting is not feasible: splenectomy or partial splenectomy 4, 5

4. For hypersplenism with liver cirrhosis awaiting transplantation:

   • Conservative management is preferred as hypersplenism often resolves post-transplant 1

Pitfalls and Caveats

• Splenectomy, while effective for cytopenia, may lead to serious infectious complications and portal vein thrombosis 6, 4
• Portosystemic shunts have questionable benefit for hypersplenism alone 4
• The effects of PSE may diminish over time, requiring repeat procedures 4
• Treatment should focus on managing the underlying cause of portal hypertension rather than just addressing the laboratory abnormalities 4