Positive Anti-dsDNA in Young Children with ITP: Key Considerations

Q: Positive Anti-dsDNA in Young Children with ITP: Key Considerations

Hello, thank you for your questions. <br>Here are my responses：<br>1. <br>I would like to ask, if both the bone marrow aspiration and chromosome tests are normal, but immunoglobulin infusion does not improve platelet counts—initially it was effective but later it failed to raise

When a young child tests positive for anti-dsDNA antibodies, it is important to monitor for potential signs and symptoms of systemic lupus erythematosus (SLE) or other autoimmune disorders. Key considerations include: 1. Clinical Evaluation: Conduct

Dear Dr.
Gao,
A toddler over two years old has been diagnosed with Immune Thrombocytopenic Purpura (ITP) with low platelet counts.
Bone marrow aspiration and chromosome tests have returned normal results.
Aside from bleeding spots caused by low platelet counts and instances of blood in stool and urine during severe thrombocytopenia, there are no other discomforting symptoms.
The platelet count has been below 10,000 for several months, and while immunoglobulin therapy initially raised the platelet count effectively, subsequent infusions have been ineffective.
1.
Given that both the bone marrow aspiration and chromosome tests are normal, what could be the reason for the inability of immunoglobulin infusions to raise the platelet count after an initial response?
2.
I have heard that acute ITP and chronic ITP are completely different diseases.
Acute ITP is often caused by viral infections and usually resolves quickly, while chronic ITP is associated with autoimmune diseases.
However, chronic ITP typically does not present with extremely low platelet counts (usually between 20,000 and 50,000).
Could our case be classified as chronic ITP? Is it possible for chronic ITP to have platelet counts below 10,000 (with a minimum of 1,000) persisting for several months?
3.
Are there similar cases of toddlers and treatment approaches that we can refer to? What is the typical prognosis for toddlers with severe thrombocytopenia due to ITP?
4.
The anti-dsDNA test result is positive, but it is the only positive result.
Could this indicate the presence of systemic lupus erythematosus (SLE), or is it merely a transient inflammatory phenomenon?
5.
Is there a possibility for the anti-dsDNA result to turn negative?
Thank you.

Elsa, 0~9 year old female. Ask Date: 2022/08/25

Dr. Gao Jiankai reply Internal Medicine

Hello, thank you for your questions.
Here are my responses:
1.
I would like to ask, if both the bone marrow aspiration and chromosome tests are normal, but immunoglobulin infusion does not improve platelet counts—initially it was effective but later it failed to raise the platelet levels—what could be the reason? --> The immune system may still have abnormalities that cause the destruction of platelets to occur at a rate higher than their production, which is why the platelet count remains low.
A detailed examination of the immune system is still necessary.
However, the effects of immunoglobulin infusion should be observed over a period of time, as it may not show immediate results.
I suggest you continue to monitor the situation or discuss it further with your physician.
2.
I have heard that acute ITP and chronic ITP are completely different diseases.
Acute ITP is often caused by viral infections and usually resolves quickly, while chronic ITP is related to autoimmune diseases.
However, chronic ITP typically does not present with extremely low platelet counts (usually between 20,000 to 50,000).
Could we be classified as having chronic ITP? Is it possible for chronic ITP to have platelet counts below 10,000 (with a minimum previously recorded at 1,000) for several months? --> I have less experience with ITP in children, but the prognosis is generally better than in adults.
The definition of chronic ITP in children is suspected when ITP persists for more than a year, so I recommend continued monitoring.
3.
Are there similar cases in young children and treatment options available for reference? What is the typical prognosis for young children with severely low platelet counts due to ITP? --> As mentioned above.
4.
The anti-dsDNA test result is positive, but it is the only positive result.
Could this indicate lupus erythematosus, or is it just a transient inflammatory phenomenon? --> It could be temporary.
The diagnosis of lupus requires additional factors and cannot be based solely on the dsDNA result.
5.
Is it possible for anti-dsDNA to turn negative? --> Yes, it is possible.
These months are still an observation period, and I recommend discussing this with your attending physician.
Department of Allergy, Immunology, and Rheumatology, Changhua Hospital, Ministry of Health and Welfare, cares about you.

Reply Date: 2022/08/25

More Info

Understanding the relationship between positive anti-dsDNA antibodies and Immune Thrombocytopenic Purpura (ITP) in young children is a complex issue that requires careful consideration of various factors. Here, I will address your questions based on the current medical understanding of ITP, autoimmune disorders, and the implications of positive anti-dsDNA results.

1. Why might immunoglobulin infusions initially raise platelet counts but later become ineffective?
The initial effectiveness of immunoglobulin therapy in raising platelet counts in ITP can be attributed to its ability to modulate the immune response. Immunoglobulins can help neutralize the antibodies that are targeting platelets, thus allowing for an increase in platelet production and survival. However, over time, the underlying autoimmune process may persist or evolve, leading to the production of new antibodies or a change in the immune system's response. This could explain why the treatment becomes less effective after an initial positive response. Additionally, the severity of the autoimmune condition may require more aggressive treatment options if standard therapies fail.

2. Is this case indicative of chronic ITP, and can chronic ITP present with extremely low platelet counts?
Chronic ITP is typically defined as a condition lasting more than six months, and it can indeed present with very low platelet counts, including counts below 10,000. While it is more common for chronic ITP to have platelet counts in the range of 20,000 to 50,000, there are exceptions. In some cases, particularly in children, chronic ITP can manifest with severe thrombocytopenia. Your child's situation, with prolonged low platelet counts and the absence of other underlying conditions, suggests that this could be a case of chronic ITP, especially given the persistence of symptoms over several months.

3. Are there similar pediatric cases and what is the prognosis for severe ITP?
There are documented cases of severe ITP in young children, although they are less common. The prognosis for children with severe ITP varies widely. Some children may experience spontaneous remission, while others may require ongoing treatment. The use of second-line therapies, such as thrombopoietin receptor agonists or immunosuppressive agents, may be necessary if first-line treatments fail. The overall prognosis is generally favorable, but it can depend on individual factors, including the response to treatment and the presence of any associated autoimmune conditions.

4. Could a positive anti-dsDNA result indicate lupus, or is it merely a transient inflammatory response?
A positive anti-dsDNA test is often associated with systemic lupus erythematosus (SLE), particularly in the context of other clinical symptoms. However, it is important to note that a positive result alone does not confirm lupus, especially if it is the only abnormal finding. In children, transient elevations in anti-dsDNA can occur due to various factors, including infections or other inflammatory processes. Therefore, further evaluation by a pediatric rheumatologist or immunologist may be warranted to assess the significance of this finding in the context of your child's overall health.

5. Is it possible for anti-dsDNA levels to become negative over time?
Yes, anti-dsDNA antibodies can fluctuate and may become negative over time, especially if the underlying inflammatory or autoimmune process resolves. In some cases, children may experience a decrease in antibody levels as their immune system stabilizes or as they respond to treatment. Regular monitoring and follow-up with a healthcare provider are essential to track these changes and adjust treatment as necessary.

In conclusion, the interplay between ITP and positive anti-dsDNA antibodies in young children is multifaceted. It requires a comprehensive approach that includes monitoring platelet counts, evaluating the significance of autoimmune markers, and considering the potential for other underlying conditions. Collaboration with a pediatric hematologist and possibly a rheumatologist will be crucial in managing your child's condition effectively. Regular follow-ups and a tailored treatment plan will help ensure the best possible outcomes.

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