Polycythemia vera and toe pain

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One of my patients have recently been diagnosed with polycythemia vera jak 2. She has not other relevant diseases. She is 78 years of and in good health.

I have been treating her routinely for years with no abnormal presentations. Recently she presented to me with an extremely painful apex of left hallux. The pain was so severe that she is unable to tolerate the weight of her bed sheet or wear occlusive footwear. Initially there was nothing to observe on the toe but after three months of pain, the apex of the toe has a 2x2mm erythematous area. Other than a change in colour of the skin, there are no other changes.

I have done a little research on polycythemia vera jak 2 and due to the hyperviscosity of the blood I suspect a microvascular throbosis.

Has anyone out there seen anything like this before?

Julie

 

Indeed, erythromelalgia does seem to occur in cases of polycythaemia vera, and from my researches it seems it can precede the appearance of other PV symptoms by several years.

But don't ignore the possibility that someone with EM might be cooling their feet to gain some relief from the pain of EM, and thus causing chilblains.

Just to balance the view, some forms of EM are being pinned on an SCN9A channelopathy.

 

Polycythemia vera

Gout is a typical presentation especially in the elderly with PV. Check for gout. Also, rouleaux is often seen in poorly managed PV. Tx is to phlebotomize down to 42/14 males or 38/12 females, asa and hydroxyurea or interferon.

Robert List owner mpdsupport.org
I'm not a doctor

From our www.mpdsupport.org archives from 1995, pearls of wisdom by
the late Dr. Gilbert

Dear Members: For your information re uric acid in MPD: Uric acid is a
by-product of the metabolism of nucleoproteins. These are molecules
that arise from DNA and RNA released by cells when they die. Patients
with MPD turnover a lot of bone marrow cells when they are in a
proliferative phase and, thus, produce a lot of uric acid.

Uric acid is cleared from the blood by the kidney. When levels of uric
acid are high in the plasma there may be crystallization of uric acid
in the joints. This is what produces gout. When a lot of uric acid has
to be cleared by the kidney uric acid may crystallize in the kidney
tubules, causing kidney stones or sludge that may damage the kidney.

In patients who have a lot of uric acid production it is advisable to
interrupt uric acid metabolism at the step before it is produced from
a precursor called hypoxanthine. This is a more soluble molecule that
does not precipitate in joints or kidneys and is cleared more readily
by the kidney.

Uric acid levels may be measure in the blood. If they are elevated
there is clearcut evidence that uric acid production is increased.
However, even if the plasma level is not increased, uric acid
production may still be increased. This can be detected by measuring
the total uric acid that appears in the urine.

I generally use a drug - Allopurinol (Zyloprim) - that inhibits the
step the turns hypoxanthine into uric acid. I use this
prophylactically in patient with MPD. Side effects are unusual and
occur early in its administration. If it is well tolerated I suggest
that the patient take one pill/day throughout the course of their
disease.

You might want to ask your doctor about this medication. Once gout has
occurred, it must be treated with colchicine and anti-inflammatory
drugs, since uric acid is already crystallized in the joint.

Dr. Gilbert

 

Gout dosen't occur in the apex of the hallux.

 

Julie, good chance the thrombosis is the cause of the pain due to the localized tissue/nerve injury from lack of bloodflow/nutrition.

Agree with Davinci, Gout occurs in joints and severe pain from Gout does not last three months.

Difficult to treat. One pearl is to use 2%Nitroglycerin ointment along base of great toe or along tarsal tunnel area. Apply thin amount with applicator twice a day. It helps to vasodilate the digits. Your using a very small amount; I never had a problem with patient having cardiac problems from it. I have also done Posterior Tibia blocks with Bupivicane as it causes greater vasodilatation and can help in breaking the pain cycle.

You may want to look into Neurontin as well. Difficult case. Hope you ascertain exact diagnosis (perhaps refer her out for a vascular consult) and that she gets better.

Steven