the Risks of Splenectomy for Severe Thrombocytopenia - Rare Disease

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Mother with severe thrombocytopenia?


Dear Dr.
Lin,
I previously inquired about my mother's low platelet count issue.
She is currently receiving treatment in the Hematology and Oncology Department at Chang Gung Memorial Hospital and is on steroid therapy.
However, the treatment has not been effective as she has reduced her steroid intake from twelve pills a day to six pills a day.
When she was taking twelve pills, her platelet count increased to over 40,000, but due to significant side effects, she was uncomfortable, leading the doctor to lower the dosage to six pills a day, resulting in a platelet count of around 20,000.
My mother has been on steroids continuously for over a year, and the doctor mentioned that long-term steroid use is not advisable as it could lead to other health issues.
Therefore, the doctor has suggested a splenectomy.
However, my mother is very concerned about whether removing the spleen will cause future immune problems.
For instance, will she be more susceptible to infections like colds, fevers, gastroenteritis, or influenza? Will her risk of severe infections or prolonged recovery times be greater than that of others after the spleen is removed?
Is splenectomy absolutely necessary for low platelet counts? My mother is very worried about this issue and fears that if her spleen is removed and her platelet count increases, her immune system might weaken, leading to frequent hospital visits.
This has caused her significant anxiety, and she is still unable to decide whether to proceed with the surgery.
I am also quite concerned as she has been experiencing multiple bruises all over her body, varying in size.
Thank you, Dr.
Lin, for your patient responses.
I appreciate your help.

A Kai, 20~29 year old female. Ask Date: 2012/09/27

Dr. Lin Sixie reply Rare Disease


Hello, Akai: ITP, or Immune Thrombocytopenic Purpura, is an autoimmune disease characterized by the immune system producing antibodies that attack platelets, leading to accelerated platelet destruction.
When platelet counts fall below 50,000, there is an increased risk of bleeding; at counts below 20,000, there is a potential risk of intracranial and gastrointestinal bleeding.
The primary medication for treating ITP patients is corticosteroids.
If symptoms are difficult to control, if thrombocytopenia persists for over a year, or in cases of severe bleeding, splenectomy should be considered.
There is also a new medication, eltrombopag, which can increase platelet counts and is used as a second-line treatment.
However, this medication is quite expensive, costing 1,344 NTD per tablet.
Currently, health insurance coverage is only available for adult chronic ITP patients who have undergone splenectomy and have failed other treatments (such as corticosteroids and immunoglobulins).
You may want to consult your physician about the possibility of splenectomy, which can effectively increase platelet counts, although patients may be at a higher risk of infection.
It is recommended to receive the pneumococcal vaccine.

Reply Date: 2012/09/27

More Info


Understanding the risks associated with splenectomy, particularly in the context of severe thrombocytopenia, is crucial for patients and their families. Your concerns regarding your mother’s condition and the potential implications of undergoing splenectomy are valid and deserve thorough exploration.

Severe thrombocytopenia, defined as a platelet count below 20,000 to 30,000 platelets per microliter of blood, can lead to significant health risks, including spontaneous bleeding and bruising, as you have observed with your mother. The spleen plays a critical role in filtering blood and managing the immune response. In cases of thrombocytopenia, particularly when caused by conditions such as immune thrombocytopenic purpura (ITP), the spleen may be overactive in destroying platelets. This is where splenectomy, or surgical removal of the spleen, comes into consideration as a treatment option.

Your mother’s experience with corticosteroids, which are often used to manage ITP, highlights a common challenge: while they can temporarily increase platelet counts, long-term use can lead to significant side effects, including weight gain, osteoporosis, diabetes, and increased susceptibility to infections. Given that your mother has been on corticosteroids for over a year, the recommendation for splenectomy may stem from the need to reduce these side effects while addressing the underlying issue of low platelet counts.

However, it is essential to understand the implications of splenectomy on immune function. The spleen is integral to the body’s immune defense, particularly against encapsulated bacteria such as Streptococcus pneumoniae and Neisseria meningitidis. After splenectomy, patients are at an increased risk for infections, particularly from these organisms. This condition is known as overwhelming post-splenectomy infection (OPSI), which can be life-threatening. Therefore, it is crucial for patients who have undergone splenectomy to receive appropriate vaccinations prior to the procedure, including pneumococcal, meningococcal, and Haemophilus influenzae type b vaccines. Additionally, prophylactic antibiotics may be recommended for a period following the surgery.

Regarding your mother’s concerns about her immune system post-splenectomy, it is true that she may experience a heightened risk of infections. However, many patients live healthy lives after splenectomy, especially with proper medical guidance and preventive measures. It is also important to note that while splenectomy can improve platelet counts significantly, it does not guarantee that the immune system will be severely compromised. Many patients adapt well and maintain a good quality of life.

The decision to proceed with splenectomy should be made collaboratively between your mother, her hematologist, and possibly an infectious disease specialist. They can provide a comprehensive assessment of her overall health, the risks versus benefits of the surgery, and the necessary precautions to take post-operatively. It is also essential to explore all treatment options, including potential newer therapies for ITP that may not involve surgery.

In summary, while splenectomy can be an effective treatment for severe thrombocytopenia, it does carry risks, particularly concerning immune function. Vaccination and preventive measures can mitigate some of these risks. Open communication with her healthcare team will be vital in making an informed decision that aligns with your mother’s health goals and concerns. Encourage her to voice her fears and questions during her consultations, as understanding the full scope of her condition and treatment options can help alleviate some of the anxiety surrounding this decision.

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