How is hypersplenism managed?

Management of Hypersplenism

The management of hypersplenism should be tailored to the underlying cause, with correction of low platelet counts generally not recommended before low-risk procedures but necessary before high-risk procedures or in the presence of bleeding.

Understanding Hypersplenism

• Hypersplenism is common in patients with advanced cirrhosis and portal hypertension, affecting all hematological cell lines, with thrombocytopenia being the most common manifestation 1
• The etiology is multifactorial, including portal hypertension with intra-splenic sequestration, myeloid toxicity (alcohol, antiviral medications, chemotherapy), anti-platelet antibodies, and/or low levels of thrombopoietin 1
• A low platelet count should be evaluated within the broader context of cirrhosis affecting hemostasis, as it alone does not necessarily predict bleeding risk in cirrhotic patients 1

Diagnostic Approach

• Rotational thromboelastometry may be superior to routine coagulation laboratory tests for accurately predicting bleeding risk during high-risk procedures, including liver transplantation 1
• In cases of splenic vein thrombosis, diagnostic procedures should include computed tomography scan, hepatic ultrasonography, angiography, and bone marrow biopsy 1
• JAK2V617F mutation determination can be helpful diagnostically in cases of Budd-Chiari syndrome and portal vein thrombosis 1

Management Strategies

Conservative Management

• As a rule, correction of low platelet count is not recommended before low-risk procedures 1
• Blood products should be used sparingly to avoid side effects, including fluid overload 1
• Management of platelets should integrate correction of coagulation factors 1

Pharmacological Interventions

• Before high-risk procedures (including transplantation) or in the presence of bleeding:
  • Thrombopoietin receptor agonists (avatrombopag and lusutrombopag) should be considered as they are superior to no treatment in avoiding platelet transfusion and rescue therapy 1
  • These agents need a few days to work and are more suited for planned procedures than emergency interventions 1

Interventional Procedures

• Partial splenic embolization (PSE) is an effective alternative to splenectomy:

  • PSE can effectively reduce hypersplenism by decreasing splenic volume and portal pressure 1
  • Studies show PSE can result in resolution of hemorrhage without recurrence in patients with gastric varices and splenic vein occlusion 1
  • When combined with other therapies like transjugular balloon-occluded retrograde transvenous obliteration (BRTO), PSE significantly reduces the risk of esophageal variceal aggravation 1
  • Common side effects include abdominal pain and subfebrility for approximately 2 weeks post-procedure 2

• Splenic vein recanalization:

  • In cases where splenic vein occlusion is the etiology of sinistral hypertension, restoration of patency corrects the underlying problem 1
  • Transjugular recanalization with angioplasty or stenting has shown resolution of upper GI bleeding without recurrence 1

Surgical Options

• Splenectomy:

  • Most effective for treating hypersplenism but associated with significant risk of portal vein thrombosis 3
  • Historically performed as standard treatment for gastric variceal bleeding with sinistral portal hypertension 1
  • Studies show 100% success rate in managing bleeding without recurrence in patients with splenic vein occlusion 1

• Shunt procedures:

  • Both shunt and non-shunt operations effectively relieve hypersplenism 4
  • Proximal splenorenal shunt not only relieves hypersplenism but also addresses complications of underlying portal hypertension 4
  • Portosystemic shunts have questionable benefit for hypersplenism alone 3

Special Considerations

• After liver transplantation, portal pressure decreases rapidly, and thrombopoietin levels increase starting from day 1, with platelet counts usually normalizing within 2 weeks 1
• In patients with pre-transplant splenomegaly, subclinical hypersplenism may persist 1
• Patients with hypersplenism who have defects in all three blood cell lineages have significantly elevated portal pressures and increased risk of complications including variceal bleeding, portal biliopathy, and gastropathy 4

Treatment Selection Based on Clinical Context

• For patients with cirrhosis without bleeding or need for high-risk procedures:

  • Observation is appropriate as hypersplenism is often of little clinical consequence 3

• For patients requiring high-risk procedures or with active bleeding:

  • Platelet transfusion can work synergistically with local hemostatic means 1
  • Consider thrombopoietin receptor agonists for planned procedures 1

• For patients with severe hypersplenism affecting quality of life or requiring bone marrow-suppressive medications:

  • Consider partial splenic embolization as a less invasive alternative to splenectomy 1, 2
  • Surgical options may be considered when less invasive approaches fail 4