Childhood thrombocytopenic purpura (ITP)
Hello Dr.
Hsiao: Regarding the previous question #185662, my family member's daughter is over two years old and has been in a state of extremely low platelet count (below 10,000) for the past six months.
After undergoing a bone marrow aspiration and chromosome testing, which confirmed normal results, she was diagnosed with immune thrombocytopenic purpura (ITP).
However, the immunological test for anti-dsDNA came back positive (this is the only positive result).
I would like to ask if there is a correlation between low platelet count and positive anti-dsDNA? If this test is positive, should we suspect other diseases? I understand that anti-dsDNA is one of the indicators of certain autoimmune diseases, but could this potentially cause such a low platelet count (below 10,000)? Is it possible for the anti-dsDNA marker to turn negative? I have seen very few cases of young children with acute thrombocytopenia below 10,000 persisting for six months, so I would like to know if this situation is considered rare? What is the likelihood of recovery in the future for children with ITP who require second-line treatment?
Elsa, 0~9 year old female. Ask Date: 2022/08/24
Dr. Xiao Kaiwen reply Pediatrics
Anti-dsDNA is indeed associated with certain autoimmune diseases, most commonly systemic lupus erythematosus (SLE).
The levels of anti-dsDNA often correlate clearly with disease activity.
However, determining whether there is a connection between your child's thrombocytopenia and positive anti-dsDNA is more challenging.
It would be advisable to discuss the possibility of other autoimmune diseases with your pediatric hematologist.
If a connection is established, improvement in the condition should lead to normalization.
Your child's situation is indeed rare in immune thrombocytopenic purpura (ITP).
If first-line treatments are ineffective, second-line targeted therapies such as rituximab can be considered, with approximately 60% of patients showing initial effectiveness, and 30% achieving complete response within a year.
However, about half of the patients may experience a relapse within four years.
Reply Date: 2022/08/24
More Info
Immune Thrombocytopenic Purpura (ITP) in toddlers is a complex condition that can be challenging to diagnose and manage, especially when accompanied by other immunological markers such as a positive anti-dsDNA test. Your inquiry about the relationship between low platelet counts and the presence of anti-dsDNA is particularly pertinent, given the unique clinical scenario you described.
Firstly, it is important to understand that ITP is characterized by a low platelet count due to the immune system mistakenly attacking and destroying platelets. In children, ITP is often classified as either acute or chronic. Acute ITP typically follows a viral infection and resolves spontaneously, while chronic ITP persists for more than six months. In your case, the child has been experiencing severe thrombocytopenia (platelet count below 10,000) for six months, which is indeed less common in pediatric ITP cases.
The presence of anti-dsDNA antibodies is primarily associated with systemic lupus erythematosus (SLE) and other autoimmune disorders. While a positive anti-dsDNA test can indicate an underlying autoimmune process, it does not directly cause low platelet counts. However, it raises the suspicion of a possible autoimmune condition that could be contributing to the thrombocytopenia. In such cases, it is crucial to evaluate the child for other autoimmune diseases, particularly if there are clinical signs or symptoms suggestive of such conditions, like rashes, joint pain, or systemic symptoms.
Regarding the possibility of the anti-dsDNA test becoming negative, it is indeed possible for antibody levels to fluctuate over time. In some patients, particularly those who respond well to treatment or whose disease activity diminishes, anti-dsDNA levels can decrease or even become undetectable. This variability underscores the importance of continuous monitoring and reassessment by a pediatric hematologist or immunologist.
As for the treatment of ITP in children, especially when first-line therapies (such as corticosteroids or IVIG) are ineffective, second-line treatments may be considered. These can include medications like rituximab or thrombopoietin receptor agonists. The likelihood of recovery with second-line treatments varies; studies suggest that approximately 60% of patients may respond initially, with about 30% achieving a complete response over time. However, it is crucial to note that there is a risk of relapse, with about half of the patients experiencing a recurrence of low platelet counts within four years.
In summary, while the relationship between low platelet counts and a positive anti-dsDNA test is not straightforward, it is essential to consider the broader clinical picture. Close collaboration with a pediatric hematologist is vital for ongoing evaluation and management. They can help determine if further investigations are warranted to rule out other autoimmune conditions and guide treatment decisions. The situation you described, with persistent severe thrombocytopenia in a toddler, is indeed less common and warrants careful monitoring and potentially more aggressive management strategies.
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