Our Summary

This research paper is a review of treatments for myelofibrosis, a type of chronic blood cancer that can cause the spleen to become unusually large (a condition known as splenomegaly). Currently available treatments don’t always shrink the spleen and the effects don’t last long. Therefore, treatments specifically targeting the spleen, such as surgical removal (splenectomy) or radiation therapy (splenic irradiation), are sometimes used to reduce the symptoms.

The researchers found that removing the spleen can improve symptoms, reduce complications of high blood pressure in the liver, and lessen the need for blood transfusions. However, this surgery carries significant risks, including a high rate of complications and death both during and after surgery. Radiation therapy can reduce the size of the spleen but the effects don’t last long and it can suppress the bone marrow, which is responsible for producing blood cells.

The researchers conclude that these spleen-targeting treatments should only be considered for severe symptoms and complications of an enlarged spleen after carefully evaluating the associated risks. They also note that these treatments may not extend the patient’s life. The development of treatments that directly target and reverse the underlying causes of myelofibrosis is necessary to significantly change the course of the disease.

FAQs

  1. What is the purpose of splenectomy and splenic irradiation in treating myelofibrosis?
  2. What are the benefits and risks associated with splenectomy in myelofibrosis patients?
  3. Do spleen-directed therapies impact the overall survival rate of patients with myelofibrosis?

Doctor’s Tip

One helpful tip a doctor might tell a patient about splenectomy is to carefully consider the risks and benefits before undergoing the procedure. While splenectomy can improve symptoms of splenomegaly and decrease complications, it also carries a significant risk of morbidity and mortality. Patients should discuss all options with their healthcare provider and make an informed decision based on their individual circumstances.

Suitable For

Patients with myelofibrosis who have massive splenomegaly and do not respond well to currently approved medical therapies may be recommended for splenectomy. This procedure can help improve symptoms of splenomegaly, decrease complications of portal hypertension, and reduce transfusion dependence. However, it is important to consider the significant peri-operative and long-term morbidity and mortality associated with splenectomy.

Splenectomy may be considered for patients with refractory symptoms and complications of massive splenomegaly after carefully weighing the risks and benefits. Additionally, splenic irradiation may be used as an alternative spleen-directed therapy to reduce splenic size, although it is limited by duration of response and myelosuppression.

Overall, spleen-directed therapies should be considered in myelofibrosis patients with massive splenomegaly who do not respond well to medical therapies. However, the development of medical therapies that target and reverse the underlying disease pathophysiology is necessary to have a significant impact on the natural history of the disease process.

Timeline

Before splenectomy:

  1. Patient presents with symptoms of myelofibrosis, such as fatigue, weakness, and abdominal discomfort.
  2. Diagnostic tests, such as blood tests and imaging studies, confirm the presence of massive splenomegaly.
  3. Patient undergoes evaluation by a hematologist to discuss treatment options, including splenectomy.
  4. Risks and benefits of splenectomy are discussed with the patient, and informed consent is obtained.
  5. Pre-operative assessment and preparation are completed, including blood tests, imaging studies, and consultation with other specialists if needed.

After splenectomy:

  1. Patient undergoes splenectomy surgery, which may be performed laparoscopically or through an open procedure.
  2. Post-operative recovery period includes pain management, monitoring for complications, and gradual resumption of normal activities.
  3. Patient experiences improvement in symptoms related to splenomegaly, such as decreased abdominal discomfort and improved energy levels.
  4. Patient is at risk for complications such as infection, bleeding, and thrombosis, and requires close follow-up with their healthcare team.
  5. Long-term effects of splenectomy include increased risk of infections and thrombotic events, which may require preventive measures such as vaccinations and blood thinners.
  6. Patient may require ongoing medical management for myelofibrosis, including medications to control symptoms and complications of the disease.

What to Ask Your Doctor

  1. What are the potential benefits of splenectomy for myelofibrosis in my case?
  2. What are the risks and potential complications associated with splenectomy?
  3. How long is the recovery period after splenectomy?
  4. Will I need any additional treatments or medications after splenectomy?
  5. How will splenectomy affect my overall quality of life?
  6. Are there any alternative treatments to splenectomy that I should consider?
  7. How often will I need follow-up appointments after the procedure?
  8. What is the long-term outlook for my condition after undergoing splenectomy?
  9. What are the potential risks and benefits of splenic irradiation as an alternative to splenectomy?
  10. Are there any clinical trials or new treatments for myelofibrosis that I should be aware of before making a decision on splenectomy?

Reference

Authors: Sankar K, Pettit K. Journal: Curr Hematol Malig Rep. 2020 Oct;15(5):391-400. doi: 10.1007/s11899-020-00598-x. PMID: 32827272