Childhood ITP treated with no symptoms and low platelets in adulthood

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Dear Doctor,

My husband has had low platelet count since his childhood and he has lived with it without any other major health problems. He remembers taking Prednisone for a while when he was a kid, and did some bone marrow test where his pediatrician confirmed he had ITP. He also has a vague idea that his platelet count was around 55K then. He is now 33 and was overall healthy except for occasional cold or fever; hence never did any blood works as an adult.

About a month ago, he slipped from few stairs and hit his right ankle. We went to a clinic and took xrays - everything was fine. But due to severe pain in calf, 2 weeks later he went to another hospital. The scanning showed internal bleeding in the calf area. At the hospital, hematologist checked his blood platelet count and it was too low, at 11,000 (on 20 Aug 2013). Immediately he was given IVIG - 3 bottles and in few days, the platelet count raised upto 96K (as of last monday, 26Aug). He is also on medication - Prednisone 60mg/day since 2 weeks.

As of 1st September, his blood platelet count went down drastically to 26K and he is advised to take another dose of IVIG immediately. His doctor has plans to take him out of Prednisone gradually due to its side effects. The doctor suggests getting a bone marrow test and splenectomy as a long term solution for the low platelet count.

He never had any health issues so far even though he always had low platelet count. So should we rush into splenectomy and the bone marrow test? How critical is the a platelet count as low as 20-30K or even lower? Is it safe to take IVIG again? If the platelet count again drops after the next IVIG dosage, what should be the next treatment? Is splenectomy really required, is it a major surgery, how effective is that to cure ITP and does it have any side effects? Are there any natural ways to increase platelet count? Should we be really worried as he always lived normal with low platelet count; is it necessary to treat ITP? How risky is to live with low platelet count; and what would be an ideal platelet count for a person with ITP?

Please advise!

Thanks,
Ragi

Submitted: 4 Days
Category: Hematologist

Expert:  Dr. Prasad Eswaran replied 4 Days.

Hello Radi,

Thanks for the query at DoctorSpring.

I understand that your husband has been suffering from low platelet count (thrombocytopenia) due to possible ITP.

In regard to your question, the normal platelet count ranges from 150000 to 450000 /ul.
If the level falls 100000/ul it is termed as thrombocytopenia. However levels than 50000/ul causes posttraumatic bleeding and less than 20000/ ul causes spontaneous bleeding, fundal and intracranial bleeding. So with his present level less than 30000, there are risks of bleeding even following a minor trauma. Luckily the bleed he had in calf is not very harmful, but that might not be the case every time. For example a bleeding in the brain can be catastrophic.

So should we rush into splenectomy and the bone marrow test? – You should go for the bone marrow test as soon as possible.  This is to rule out any other condition that may cause low platelets and will help in forming a final diagnosis of ITP. You need not ‘rush’ into splenectomy, but it needs to be considered as your husband is not having persistent response with Prednisolone, IVIG or having a spontaneous remission. Ideally one can wait 4-6 weeks before splenectomy to see whether the counts return normal. But in your husband’s scenario, I would recommend to trust your Doctors judgement. With the limited information I also feel that splenectomy is the best way to move forward. Other second line options are not as effective as splenectomy. When done electively this is a safe procedure.  

Splenectomy acts by removing the major site of platelet destruction and brings down the concentration of circulating antiplatelet antibodies. It is a safe procedure. Side effects like infections (OPSI), fever may occur in certain individuals, but they are manageable. Your Doctor will recommend some immunization prior to the procedure. IVIG can be used but provide only temporary effect. So it would be best to go for a long-term solution. The count should be near normal. At least should be above 50,000 to prevent chances of bleed.

Hope this helps.

Please feel free to ask followup questions via email itself.

Thank you.

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Patient replied :

Dear Doctor,

 

Thank you for your detailed reply. This has been very informative and answered many of our concerns. We really appreciate you for taking time and looking into our case. 

 

My husband did a blood test 2 days back and I'm attaching the results in this mail. Can you please review this and share your expert opinions? The comments in the report says many of the results are outside normal range and need to consult a doctor at the earliest. Are any of the counts abnormal due to taking Prednisone? Is there anything really alarming in this result?

 

He took another dosage of IVIG yesterday, and one more in scheduled today. His doctor has asked for a blood test on Friday. 

 

Is bone marrow test a complicated process? Are there any side effects for taking this test?

 

Looking forward to hear from you.

 

Thanks a lot!

 

Regards


Expert:  Dr. Prasad Eswaran replied 3 Days.

Hello, 

 

Do you have any of his previous blood test value with you ? - Especially of the WBC count.

Also if you have a "Peripheral Smear Report" please share with me. 

 

Thank you

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Patient replied :

Hello Doctor,

 

I've attached 2 blood reports, done on 26th Aug and 1st Sep. A blood test was done on 21st Aug, but I don't have a copy with me right now. I remember when he was hospitalized (20Aug - 22Aug), the doctor mentioned that the WBC count was high and a possible reason for that could be the internal bleeding in the calf area. He hasn't done any blood tests in the last 15-20 years other than these tests done last month. We do not have any peripheral smear report too.

 

Regards


Expert:  Dr. Prasad Eswaran replied 2 Days.

Hello,

 

Thank you for the additional details. 

 

The reports in general shows two abnormalities. 1.Low platelets (which we already know) 2.High WBCs.

WBCs are supposed to go up when there is an infection or inflammation. It is their function. So we cannot call it exactly 'abnormal'. Even drugs like prednisone can cause this. A peripheral smear should have been done already. If you are under the care of a Hematologist, he/she might have observed seen it directly under microscope. 

 

So the low platelet count is the main concern in the blood tests. Rest can be considered as a soft finding for now. 

 

Bone Marrow test is a not a complicated procedure. Side effects are rare. You SHOULD go for it and SHOULD NOT delay it if your Doctor has recommended it. It is a very common procedure done in Haematology on a daily basis. 

 

Hope this helps

Thank you

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Patient replied :

Dear Doctor,

 

I would like to update you about my husband's treatment for ITP. As you suggested, a bone marrow test was done and its confirmed that he has ITP and other than that, everything looked normal. As of now, he is given IVIG every 2 weeks, and he is completely off Prednisone as it wasn't effective. The platelet count goes upto 90-95000/uL soon after IVIG is given and the next week it goes down to 50-60000/uL, and 2 weeks later it comes down to 25000/uL. Last week it even went down to 19000/uL. 

 

His doctor now advises to go with the next level of treatment - taking 4 dosages of Rituximab. He mentioned if Rituximab is not effective, he'll have to go with splenectomy. May I know how effective would be the treatment of Rituxin? Hematologist also mentioned that my husband will be given Hep C shots and shingles vaccine. Last week, a test for Hep B was done and it came out normal. Internet sources say there are many possible side effects for Rituxin, so I'm a bit concerned. Can you please share your expert opinion, doctor?

 

Please find attached the CBC results for your reference. We greatly look forward for your thoughts.

 

Thank You


Expert:  Dr. Prasad Eswaran replied 1 Day.

Hello,

 

Thank you for the followup.

 

Rituximab is a second line drug. So naturally the side effects might be bit more than traditional drugs ( that is why it is kept as second line). However this should not deter you from making a decision. Side effects can occur even with simple pain medication, so there is no logic in being overly concerned. 

 

Rituximab can be effective upto 40% of patients. However the issue is how long the effect will last. Usually splenectomy is considered in line with Rituximab. (Not always like Splenectomy if Rituximab fails, although nothing wrong in this approach) . So one consideration here is where he should go for Splenectomy straight away. This I will not be able to make any recommendation, only your treating Doctor can. So please discuss this with your Doctor .

 

Thank you

Wish you good health

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