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What Causes Polycythemia Vera?
Question: My grandson is 24 and has autism. learning disabilities, epilepsy not sure if these have any relation to my daughters MND Over the last 3/4 years he has been diagnosed with polycythemia and frequently has to have a pint of blood taken from him. All our relations on both sides are white British, can you explain the disease to me and will he always have it or is there some cure?
Brief Answer:
We can avoid complications to some extent, but can't cure 100%!
Detailed Answer:
Thanks for consulting me at HCM . Your trust in HCM means a lot to me as a Physician.
I have completely reviewed your health query, and can well realize all those concerns. Being your physician, I will take care all of them today and will give extend my expertise to solve the problems.
Polycythemia Vera (PV) is a Genetic disease of the stem cells (present in the bone marrow). There are also normal stem cells present, but the deformed cells due to some mutations in genes and unknown origin of proliferation defect in stem cell lines cause multiple folds increased production of red blood cells, white blood cells and platelets. Red blood cells are produced more and more, just like a cancerous growth, they are uncontrolled.
It is a disease of people from any descent and any ethnic group, of any age group or gender.
We as Physicians focus on the fact that there is no thrombotic events in the blood vessels which are common in such patients. So we have to keep his Hematocrit below 45%. That's why it is major part of treatment that we draw periodically some blood out of his veins as he has much larger mass of red blood cells and that is not compatible for normal functioning of the body. If blood counts are out of control we may go for giving Hydroxyurea OR Interferon-alpha.
Other treatment options includes Aspirin. If there are spleen issues like infarctions or spleen enlarges in size we need to go for surgical removal of the spleen.
Bone marrow complications:-
Bone marrow may become just a fibrous tissue (fibrotic changes) or it may transform into a malignant transformation (Leukemia). We need to keep an eye on peripheral blood and if suspicion, we may go and test his bone marrow through biopsy.
Unfortunately there is no definitive cure of this disease. Previously the survival was upto a maximum to 2-3 years but today more than 20 years if no major complications ensue like:
Thromboembolisms.
Organs enlarge like spleen, liver etc.
Peptic Ulcer disease (ulcers in stomach and duodenum that may bleed easily).
Bleeding anywhere in body due to blood vessel issues and viscous blood.
If the bone marrow gets fibrosed, all the cell lines get diminished supply (low Red and White blood cells and platelets).
Leukemias may also be a complication.
Final suggestion:-
Keeping in touch with a Hematologist is very necessary for periodic testing and early detection and treatment of the complications if any.
Hope I answered all of your queries. Let me know if you have further clinical concerns or doubts. I will be more than happy to get back to you with my expert opinion.
For any disease you have, a timely follow-up is very necessary regarding the treatment efficacy and to see if there is a need to change the treatment options. So keep a follow up to see how you doing (Always write down my name in start of your questions at this forum & I will be with you in no time).
Regards!
DR. HANIF
USA
We can avoid complications to some extent, but can't cure 100%!
Detailed Answer:
Thanks for consulting me at HCM . Your trust in HCM means a lot to me as a Physician.
I have completely reviewed your health query, and can well realize all those concerns. Being your physician, I will take care all of them today and will give extend my expertise to solve the problems.
Polycythemia Vera (PV) is a Genetic disease of the stem cells (present in the bone marrow). There are also normal stem cells present, but the deformed cells due to some mutations in genes and unknown origin of proliferation defect in stem cell lines cause multiple folds increased production of red blood cells, white blood cells and platelets. Red blood cells are produced more and more, just like a cancerous growth, they are uncontrolled.
It is a disease of people from any descent and any ethnic group, of any age group or gender.
We as Physicians focus on the fact that there is no thrombotic events in the blood vessels which are common in such patients. So we have to keep his Hematocrit below 45%. That's why it is major part of treatment that we draw periodically some blood out of his veins as he has much larger mass of red blood cells and that is not compatible for normal functioning of the body. If blood counts are out of control we may go for giving Hydroxyurea OR Interferon-alpha.
Other treatment options includes Aspirin. If there are spleen issues like infarctions or spleen enlarges in size we need to go for surgical removal of the spleen.
Bone marrow complications:-
Bone marrow may become just a fibrous tissue (fibrotic changes) or it may transform into a malignant transformation (Leukemia). We need to keep an eye on peripheral blood and if suspicion, we may go and test his bone marrow through biopsy.
Unfortunately there is no definitive cure of this disease. Previously the survival was upto a maximum to 2-3 years but today more than 20 years if no major complications ensue like:
Thromboembolisms.
Organs enlarge like spleen, liver etc.
Peptic Ulcer disease (ulcers in stomach and duodenum that may bleed easily).
Bleeding anywhere in body due to blood vessel issues and viscous blood.
If the bone marrow gets fibrosed, all the cell lines get diminished supply (low Red and White blood cells and platelets).
Leukemias may also be a complication.
Final suggestion:-
Keeping in touch with a Hematologist is very necessary for periodic testing and early detection and treatment of the complications if any.
Hope I answered all of your queries. Let me know if you have further clinical concerns or doubts. I will be more than happy to get back to you with my expert opinion.
For any disease you have, a timely follow-up is very necessary regarding the treatment efficacy and to see if there is a need to change the treatment options. So keep a follow up to see how you doing (Always write down my name in start of your questions at this forum & I will be with you in no time).
Regards!
DR. HANIF
USA
Above answer was peer-reviewed by :
Dr. Arnab Banerjee
Many thanks for your comprehensive answer, because of XXXX's other problems autism,epilepsy and learning difficulties he has a named nurse who keeps a close eye on his whole XXXXXXX
The benefits of our national health service which is still free from cradle to grave and FREE PRESCRIPTIONS! We are very fortunate but not sure for how much longer the country can continue!!!!!!!!!!!!!
XXXXXXX XXXX
The benefits of our national health service which is still free from cradle to grave and FREE PRESCRIPTIONS! We are very fortunate but not sure for how much longer the country can continue!!!!!!!!!!!!!
XXXXXXX XXXX
Brief Answer:
Hope for the best and I am sure everything goes smooth!
Detailed Answer:
Welcome back Miss XXXXXXX XXXX. I feel really sorry for the situation XXXXXXX has.
Your grandson is really lucky to have these facilities otherwise most of the countries in the world are not welfare states and doesn't have such benefits to offer to their residents.
I hope everything goes smooth for XXXXXXX Just reassure that the Nurse keeps a real eye on his blood hematocrit and organs should not grow (like liver, spleen etc).
Hope I answered all of your queries. Let me know if you have further clinical concerns or doubts. I will be more than happy to get back to you with my expert opinion. Please don't forget to give Five Star rating to help me assist patients in a better way; and, please don't forget to 'Close This Discussion'.
For any disease you have, a timely follow-up is very necessary regarding the treatment efficacy and to see if there is a need to change the treatment options. So keep a follow up to see how you doing (Always write down my name in start of your questions at this forum & I will be with you in no time).
Regards!
DR. HANIF
USA
Hope for the best and I am sure everything goes smooth!
Detailed Answer:
Welcome back Miss XXXXXXX XXXX. I feel really sorry for the situation XXXXXXX has.
Your grandson is really lucky to have these facilities otherwise most of the countries in the world are not welfare states and doesn't have such benefits to offer to their residents.
I hope everything goes smooth for XXXXXXX Just reassure that the Nurse keeps a real eye on his blood hematocrit and organs should not grow (like liver, spleen etc).
Hope I answered all of your queries. Let me know if you have further clinical concerns or doubts. I will be more than happy to get back to you with my expert opinion. Please don't forget to give Five Star rating to help me assist patients in a better way; and, please don't forget to 'Close This Discussion'.
For any disease you have, a timely follow-up is very necessary regarding the treatment efficacy and to see if there is a need to change the treatment options. So keep a follow up to see how you doing (Always write down my name in start of your questions at this forum & I will be with you in no time).
Regards!
DR. HANIF
USA
Note: For detailed guidance on genetic screening consult a genetics specialist
Above answer was peer-reviewed by :
Dr. Raju A.T
Answered by
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