Our Summary

This study looks at a rare type of hemoglobin (the molecule in red blood cells that carries oxygen) called Hemoglobin Volga. This variant can cause a type of anemia where the body destroys its own red blood cells. The researchers studied two members of a Danish family who had this condition. They had their spleens removed as part of their treatment, and both experienced multiple blood clots throughout their lives. The father also had a small stroke. The son, despite blood-thinning treatment, had multiple blood clots in his youth and died from a chronic lung condition related to blood clots, combined with an infection.

The researchers found 25 reported cases of Hemoglobin Volga worldwide. Symptoms mainly relate to anemia. 15 of those patients had their spleens removed. Blood clots have been reported in only three other patients, all of whom also had their spleens removed. The father in this study is the first reported case of a person with Hemoglobin Volga who has had both types of blood clots, in the veins and arteries. The exact reason why people with Hemoglobin Volga are more prone to blood clots is not known, but it is likely related to having the spleen removed.

FAQs

  1. What is Hemoglobin Volga and how does it affect the body?
  2. What is the relationship between Hemoglobin Volga, spleen removal, and blood clots?
  3. What are the typical symptoms in patients diagnosed with Hemoglobin Volga?

Doctor’s Tip

A doctor might tell a patient who has had a splenectomy to be aware of the increased risk of blood clots, especially if they have a rare condition like Hemoglobin Volga. It is important for patients to discuss any concerns or symptoms with their healthcare provider and to follow any recommended blood-thinning treatments to help reduce the risk of complications. Additionally, patients should be vigilant about any new or worsening symptoms and seek medical attention promptly if needed.

Suitable For

Patients with conditions that cause chronic destruction of red blood cells, such as Hemoglobin Volga, are typically recommended splenectomy. This is because the spleen is responsible for removing old or damaged red blood cells from the body, and if it is not functioning properly, it can lead to severe anemia. In cases where medical treatment is not effective in managing the condition, splenectomy may be necessary to improve the patient’s quality of life and prevent complications such as blood clots.

Timeline

Before splenectomy:

  • Patients may experience symptoms of anemia, such as fatigue, weakness, and pale skin
  • Patients may undergo blood tests to diagnose the type of anemia and determine if splenectomy is necessary
  • Patients may receive blood transfusions or other treatments to manage their anemia
  • Patients may undergo imaging tests to assess the size and function of the spleen
  • Patients may receive counseling and education about the risks and benefits of splenectomy

After splenectomy:

  • Patients may experience improved red blood cell production and anemia symptoms may improve
  • Patients may be at increased risk for infections, particularly from encapsulated bacteria such as Streptococcus pneumoniae
  • Patients may need to receive vaccinations to prevent infections, including the pneumococcal vaccine
  • Patients may need to take antibiotics long-term to prevent infections
  • Patients may need to undergo regular blood tests to monitor for any complications related to the absence of the spleen
  • Patients may need to take additional precautions to prevent injury, as the spleen plays a role in immune function and in filtering out damaged red blood cells

Overall, the decision to undergo splenectomy is a complex one that involves weighing the potential benefits of improved anemia symptoms against the risks of infections and other complications associated with the absence of the spleen.

What to Ask Your Doctor

  1. What are the potential risks and benefits of a splenectomy for my specific condition?

  2. How will having my spleen removed affect my risk of developing blood clots?

  3. Are there any alternative treatments or management strategies that could help reduce my risk of blood clots without having to remove my spleen?

  4. How will the removal of my spleen impact my overall health and immune function?

  5. What follow-up care will be necessary after the splenectomy to monitor for any potential complications, such as blood clots?

  6. Are there any specific precautions or lifestyle changes I should consider after having my spleen removed to reduce my risk of blood clots?

  7. Are there any specific medications or treatments that can help prevent blood clots in individuals who have had their spleens removed?

  8. How common is it for individuals with Hemoglobin Volga to experience blood clots, and what steps can be taken to minimize this risk after a splenectomy?

  9. Are there any ongoing research studies or clinical trials investigating the link between Hemoglobin Volga, splenectomy, and blood clotting disorders that I should be aware of?

  10. How will the presence of Hemoglobin Volga impact my overall treatment plan and management of potential blood clotting complications in the future?

Reference

Authors: Breinholt JK, Glenthøj A, Bor MV. Journal: Hemoglobin. 2024 Mar;48(2):79-86. doi: 10.1080/03630269.2024.2335933. Epub 2024 Apr 2. PMID: 38565203