ITP in Children: Immune System's Role in Low Platelet Counts

Childhood thrombocytopenic purpura (ITP)

Hello Doctor: A family member's child has been diagnosed with ITP for a year.
Previously, they used Romiplostim, and the platelet count generally ranged between 20,000 to 70,000, with a maximum count reaching 170,000.
When using immunosuppressants or during periods of low immunity (or when having a cold), the platelet count tends to increase.
This can indicate that the low platelet count is caused by the immune system, correct?

Ju, 0~9 year old female. Ask Date: 2023/03/17

Dr. Xiao Kaiwen reply Pediatrics

The currently accepted reason for Immune Thrombocytopenic Purpura (ITP) is that it is caused by an abnormal immune system, which is why the use of immunosuppressants can be effective.
However, the term "immunocompromised" is somewhat vague; during this time, platelet counts may not necessarily increase or decrease.

Reply Date: 2023/03/17

More Info

Immune Thrombocytopenic Purpura (ITP) in children is a condition characterized by low platelet counts, which can lead to increased bleeding and bruising. The underlying mechanism of ITP is primarily related to the immune system's abnormal response, where the body mistakenly targets and destroys its own platelets. This autoimmune aspect is crucial in understanding the fluctuations in platelet counts observed in affected children.

In your case, the child has been diagnosed with ITP for over a year and has experienced varying platelet counts, ranging from 20,000 to 170,000. The use of immunosuppressive agents, such as sirolimus, has shown effectiveness in managing the condition, particularly when the immune system is involved in the destruction of platelets. The observation that platelet counts tend to rise during periods of immunosuppression or when the immune system is compromised (such as during a cold) supports the hypothesis that the immune system plays a significant role in the pathophysiology of ITP.

When the immune system is weakened, it may not produce as many antibodies that target platelets, leading to a temporary increase in platelet counts. This fluctuation can be interpreted as evidence that the immune system is indeed a contributing factor to the low platelet counts seen in ITP. However, it is essential to note that while the immune system's activity can explain some of the variations in platelet levels, it does not provide a complete picture of the disease. Other factors, such as infections, medications, and individual variations in immune response, can also influence platelet counts.

In children with ITP, particularly those with persistent or chronic forms of the disease, it is common to monitor for other autoimmune markers, such as anti-dsDNA antibodies, which can indicate the presence of other autoimmune conditions, like systemic lupus erythematosus (SLE). The presence of these antibodies does not directly cause low platelet counts but may suggest a broader autoimmune process that could complicate the clinical picture.

Regarding the child's condition, it is crucial to maintain regular follow-ups with a pediatric hematologist who can monitor the child's platelet counts and overall health. If the child experiences persistent low platelet counts or other concerning symptoms, further investigations may be warranted to rule out other underlying conditions or complications.

In summary, the immune system plays a pivotal role in the pathogenesis of ITP in children, and fluctuations in platelet counts can often be linked to immune activity. While immunosuppressive treatments can be effective, ongoing monitoring and a comprehensive approach to management are essential for optimizing outcomes in children with ITP.

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