EM, leukemia and blood pressure

I am just wrapping up six months of treatment for AML-MRC (acute myeloid leukemia with myelodysplasia-related changes). I had been diagnosed with EM 18 years earlier, and it had gradually improved over time, with aspirin and sandals. EM flare ups started around the time I was diagnosed, and got worse with chemotherapy (3 separate rounds of it). When I was diagnosed with EM, it was considered a blood disorder. The cardiac specialist who is treating me for chemo-related heart damage tells me it is now considered a neurological disorder. It doesn’t make much difference because the flare ups are happening less and less, and I do’t want to add any more prescription meds to those I already have to take.

The hematologist who originally diagnoses EM said it is a problem controlling blood pressure, because when it flares, the heart starts beating like crazy to send blood to the hands and feet – esp. the feet. Since I’m taking my blood pressure several times a day, I decided to check it when EM flares up. Holy cow, Nelly! My BP popped from 105/68 with a pulse of 81 to 164/85 with pulse of 99. And you can’t just down some more BP pills – that’s a good way to kill yourself. But the doctor who treats your hypertension really, really MUST know about this.

My physicians never found any blood pressure changes with my EM and when initially seeking treatment I did have some appointments where I was flaring. My blood pressure was always in a normal range.

I’ve not read there is any cardiac involvement in EM. One proposed pathophysiological pathway for the erythema seen in EM is blood shunting through arteriovenous anastomoses.

It’s a cycle that looks like this:
0-S0022202X15300312-gr1

What that means is that, rather than passing through the capillary bed for nutritive exchange, warm arterial blood is simply being shunted towards the skin. Which deprives the tissue of oxygen, leads to hypoxia, and causes more blood to shunt to the area, repeating the cycle. That short circuit exists to allow the body to rapidly cool in conditions of hyperthermia. Warm arterial blood is shunted towards the skin to radiate heat and cool the body to protect the internal organs. In EM that short circuit may be errantly used.

I assume you are not or at least at the time of testing, were not, hypertensive. I have been for many years. That is why my hematologist was concerned from the outset about controlling my high blood pressure in the face of frequent EM flares. That’s good for you, because it’s one less treatment challenge to be surmounted.

I did a quick google search and found that high blood pressure during flares is not unusual. I didn’t spend a lot of time on it, but found an article published by the National Center for Biotechnology Information associating erythromelalgia with hypertension and leukocytoclastic vasculitis (“hot hands and feet”).

Merck says erythromelalgia is a heart and blood vessel disorder. Other medical sources list erythromelalgia as neurological or as a type of fibromyalgia. What is called erythromelalgia may not be a single disorder, but a bunch of them with similar symptoms. The National Organization of Rare Disorders (NORD) says about 5% of erythromelalgia cases appear to be genetic, which seems to be the situation in my case as my mother, one of her sisters and their maternal grandmother all had erythromelalgia symptoms.

I also found that erythromelalgia is related to quite a number of other disorders, and the one that caught my attention was thrombocytosis. According to NORD, it is characterized by abnormalities of certain bone marrow (i.e., precursor) cells that produce particular blood cells. In 18 years, once I had been given a diagnosis and told that there is no treatment because it’s so rare that there is no money to be made in research, I had not done much personal research on EM. Now that I’m in remission from AML-MRC (naturally, a rare type of leukemia) and I see an association between EM and abnormalities of bone marrow cells, my antennae have gone up. I’m being treated at Virginia Commonwealth University Hospital, in a treatment program that is “investigational,” a term indicating beyond research trial but still not widely available. One of the doctors, in an early interview, was puzzled that neither I nor other family members had previously had leukemia, because it’s usually a precursor to AML-MRC, which strikes people over 60 years old. He did not seem to be familiar with EM, and since I and about half my family members have Factor V Leiden (5 times more likely than average person to get blood clots), which is not as rare as EM, he ventured that the connection was Factor V. But I’m going to call the EM, thrombocytosis and bone marrow abnormalities to my leukemia doctor’s attention. I’m sure she will be interested, and it could lead to some meaningful breakthroughs.

So, Carterdk, I thank you for your response, which got me off my duff and looking stuff up.

I hope you didn’t think I was saying you don’t have high blood pressure as part of your EM flares. I was only saying it is not necessarily a concomitant feature of erythromelalgia.

EM is more a symptom than disease for most people. Primary erythromelalgia is caused by mutation of the voltage-gated sodium channel α-subunit gene SCN9A. Secondary erythromelalgia is multifactorial. It’s possible you have familial secondary erythromelalgia associated with your Factor V Leiden mutation. Erythromelalgia associated with myeloproliferative disorders such as polycythemia vera and essential thrombocytosis are secondary. In those instances it is best to treat the underlying disorder and that will usually also treat the erythromelalgia.

Carterdk, I never took offense from your comments. I was already hypertensive, hence my doctor’s concern about the effect of EM flare ups on BP and trying to keep it within some kind of reasonable range.

This support group has prompted me to look more closely at EM, wondering if it’s a single diagnosis/disorder or a lot of different ones, and whether my EM diagnosis is correct at all. I’ve sent links and info to my oncologist/hematologist, who is a researcher as well as treatment provider, about essential thrombocythemia (thrombocytosis). It is often diagnosed as EM, and is characterized by abnormalities of certain bone marrow (i.e., precursor) cells that produce particular blood cells. This is the kind of precursor blood disorder that the doctors were looking for when I began treatment. It puzzled them that the only one I knew of was a blood clotting disorder, Factor V Leiden. So we’ll see. Maybe I can help other leukemia patients.

Sheila