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Suggest Treatment For Idiopathic Erythrocytosis

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Posted on Sun, 15 Oct 2017
Question: I'm diagnosed with idiopathic erythrocytosis. Through many negative tests with oncology and hematology, (bone marrow biopsy, heart XXXXXXX pulmonary fitness test, ehcocardiagram, sleep apnea test, thyroid panel, cbc's and so on) it still remains a mystery. I'm having a phlebotomy as needed to keep the HCT and HGN down to normal. Symptoms most of this year include daily light headedness when upright, fatigue that progresses through the day, sporadic shortness of breath, and sporadic low grade headaches. The dizziness and fatigue have increased in intensity over these months. No chest pains. I also have neuropathy in both feet since 2007, and a little more pain and discomfort each year. I've tried 6 drugs and a pain clinic...no good. I have a granddaughter who is diagnosed with POTS (blood pressure drops with the tilt table test). So I tried mine at home from a sitting position to standing. Sitting - 107/66, standing - 129/86. I have read that nerve damage with neuropathy can hamper blood flow when standing up.
I had radiation at Mayo Clinic 10 years ago ref prostate cancer (surgeon didn't get it all the first time). I am not diabetic. Never had a heart attack or stroke...always active. Quality of life has diminished considerably. Back in February my GP suggested to look into neurology if all else failed. Phlebotomy does not improve the way I feel, except maybe in the first half of the morning. Not sure where to go with this issue...maybe try Mayo if they would accept me. I know neurological disorders have a very long list.
I should also include that my wife believes that I have more difficulty in processing thoughts, memory, etc., as this year progressed. Nothing major, just little things here and there. Thanks for any input.
doctor
Answered by Dr. Ishu Bishnoi (7 hours later)
Brief Answer:
Hyperviscosity syndrome, peripheral neuropathy and dementia

Detailed Answer:
Hi xxxxxxxx, thanks for asking from HCM.


I can understand your concern. It is inspiring that despite suffering from so many illnesses, you are still active. Your most problematic illness is "idiopathic erythrocytosis". It is causing "hyperviscocity syndrome". The concentrated blood is causing headache, fatigue, shortness of breath and dizziness. You mentioned that despite repeated phlebotomies, you are not getting much relief. In such case, alternatives can be tried like

-Drink at least 3.5 to 4 litre of hypo-osmolar fluid (water, lemon water).
- Avoid going in hot, humid environment.
- Keep yourself mostly in cold environment.
- Dont exert too much. Always carry water bottle with you.
- Use ecospirin 75 to 150mg daily after proper prescription. It will reduce chances of thrombus and thus pulmonary embolism.
- For dizziness, you can take tablet betahistine after prescription.
- Avoid any kind of diuretics like furosemide, thiazides etc.

Your second problem is peripheral neuropathy which is now involving your autonomic nervous system (fall in BP after positional change). As it is present since 2007, it must have caused some permanent damage to your nerves. To prevent its progression and treat it, we must first know its cause

- Whether it was due to hyperviscocity syndrome, alcohol, age related or any other reason? Please let me know.
- What was the nerve conduction velocity report, if done any?

After knowing the cause, we must first treat the root cause. Along with it, for some recovery in nerves, you must follow few steps

- Take nerve specific vitamin like B12, folic acid, pantothenic acid, pyridoxine, benfotamine.
- Avoid any alcohol intake.
- Eat green leafy vegetables and fruits. These food items will provide both vitamin and minerals.
- Wear leg stockings to prevent sudden fall in BP.
- If there is sensory loss in feet, use soft shoes for them. It will prevent any un-noticed injury.
- If there is tingling or current/pain sensations, you can take tablet Pregabaline after proper prescription.
Symptomatic and root cause treatment will help you in getting relief in neuropathy symptoms.

Your 3rd complaint was "dementia like features". It could be due to
- Age related
- Alzheimer disease
- Diffuse cerebral atrophy
- Ishemic

Now to treat it, we need few investigations to confirm the cause. It inlcudes

- Your higher mental function evaluation. You must consult a good neurologist to find the deficit.
- MRI brain with DWI-ADC and angiography to know about ischemia, any lesion, cerebral atrophy.

Overall these two investigations mostly confirm the diagnosis. I need to know about these two. Only after that, I will be able to help you with memory problem.
Hope you got my point. If any doubt, do let me know.
Thanks. Take care.
Above answer was peer-reviewed by : Dr. Kampana
doctor
default
Follow up: Dr. Ishu Bishnoi (6 hours later)
Dr. Bishnoi:

Thank you for your very thorough response. Since I am not diabetic, radiation is suspected as a possible cause of neuropathy (32 treatments 10 years ago ref prostate cancer), but cause is unknown. I take low dose aspirin since last February as well as warfarin. My alcohol consumption is rare. I've been wearing compression socks for a couple of years (swollen ankles if I don't). Sensations in my feet include burning, sporadic sharp pain, tightness, general discomfort all the time, cold temps makes it worse. Several years ago I had a test involving the lower extremities; I think is was an EMG. No abnormalities at that time. We live in a colder climate in northern Wisconsin. I can't have an MRI due to my pacemaker (A-fib 15 years ago). My hematologist thought this might be polycythemia vera, but the bone marrow biopsy was normal. I know that there are some cases considered idiopathic in which the cause is never determined.
I would certainly consider seeking a good neurologist for future care. Recently my wife and I participated in a voluntary Alzheimer testing program here in our city and passed without a problem. I've also had a CT covering the lower abdominal area up through the head...no detected problems. This was done last May.
Thanks again.

doctor
Answered by Dr. Ishu Bishnoi (20 hours later)
Brief Answer:
History analysis and management

Detailed Answer:
Hi xxxxxxxxxxxxx, welcome back.


Thanks for providing me detailed history pf your symptoms. Radiation is a known entity which can cause permanent neuropathy. However it must not be progressive. In your case, the neuropathy is progressive and now involved autonomic nervous system. I think you must get an nerve conduction velocity done after clinical examination. It will confirm the diagnosis and tell the extant of it. To confirm further, nerve biopsy can help. It can help in finding the cause.
Your sensory symptoms are mixture of both hyper viscosity and neuropathy symptoms. When both conditions will be managed appropriately, you will get relief.

For dementia, if MRI brain can not be done, you need a detailed evaluation by neurologist. It is good that you and your wife had passed "Alzheimer testing programme". I am happy that you don't have this disease. Rest all pathologies except ischemic, can be managed well with medication. For memory and thought process improvement, you should

- Drink plenty of fluids, eat green leafy vegetables/fruits.

- Take tablet donepezil, amantadine combination for memory improvement.

- For cognitive improvement, you should take tablet citicoline and piracetam. Usually these medications are given to stroke patients who have severe cognitive function impairment. You can ask your neurologist about these medication.

- Try to play games like "Sudoku" or "Chess". I advise my patients to play these games. These games improve thought processing.

Hope it will help you. If any doubt, do let me know.
Thanks. Take care.

Above answer was peer-reviewed by : Dr. Vaishalee Punj
doctor
Answered by
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Dr. Ishu Bishnoi

Neurologist, Surgical

Practicing since :2007

Answered : 901 Questions

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Suggest Treatment For Idiopathic Erythrocytosis

Brief Answer: Hyperviscosity syndrome, peripheral neuropathy and dementia Detailed Answer: Hi xxxxxxxx, thanks for asking from HCM. I can understand your concern. It is inspiring that despite suffering from so many illnesses, you are still active. Your most problematic illness is "idiopathic erythrocytosis". It is causing "hyperviscocity syndrome". The concentrated blood is causing headache, fatigue, shortness of breath and dizziness. You mentioned that despite repeated phlebotomies, you are not getting much relief. In such case, alternatives can be tried like -Drink at least 3.5 to 4 litre of hypo-osmolar fluid (water, lemon water). - Avoid going in hot, humid environment. - Keep yourself mostly in cold environment. - Dont exert too much. Always carry water bottle with you. - Use ecospirin 75 to 150mg daily after proper prescription. It will reduce chances of thrombus and thus pulmonary embolism. - For dizziness, you can take tablet betahistine after prescription. - Avoid any kind of diuretics like furosemide, thiazides etc. Your second problem is peripheral neuropathy which is now involving your autonomic nervous system (fall in BP after positional change). As it is present since 2007, it must have caused some permanent damage to your nerves. To prevent its progression and treat it, we must first know its cause - Whether it was due to hyperviscocity syndrome, alcohol, age related or any other reason? Please let me know. - What was the nerve conduction velocity report, if done any? After knowing the cause, we must first treat the root cause. Along with it, for some recovery in nerves, you must follow few steps - Take nerve specific vitamin like B12, folic acid, pantothenic acid, pyridoxine, benfotamine. - Avoid any alcohol intake. - Eat green leafy vegetables and fruits. These food items will provide both vitamin and minerals. - Wear leg stockings to prevent sudden fall in BP. - If there is sensory loss in feet, use soft shoes for them. It will prevent any un-noticed injury. - If there is tingling or current/pain sensations, you can take tablet Pregabaline after proper prescription. Symptomatic and root cause treatment will help you in getting relief in neuropathy symptoms. Your 3rd complaint was "dementia like features". It could be due to - Age related - Alzheimer disease - Diffuse cerebral atrophy - Ishemic Now to treat it, we need few investigations to confirm the cause. It inlcudes - Your higher mental function evaluation. You must consult a good neurologist to find the deficit. - MRI brain with DWI-ADC and angiography to know about ischemia, any lesion, cerebral atrophy. Overall these two investigations mostly confirm the diagnosis. I need to know about these two. Only after that, I will be able to help you with memory problem. Hope you got my point. If any doubt, do let me know. Thanks. Take care.