In which I answer your questions (part 1)

One of the things I like about this blog is that it shows me what search terms people use that lead them to finding this blog.  So since I'm going to answer some email questions this week, I figured I'd address some of the topics people search for as well.

"Bone marrow biopsy and mcas"

This is a big one.  Mast cell patients generally get bone marrow biopsies when SM is suspected.  Most of the time, for MCAS patients, the biopsy comes back normal, which rules out SM.  However, occasionally, it comes back with some findings but not enough for an SM diagnosis.  So what do you have then?

Generally speaking, you have MMAS (monoclonal mast cell activation syndrome.)  These patients have bone marrow biopsies that are negative for dense infiltrates, but positive for CD25 receptor expression and/or the D816V CKIT mutation. 

I know of two instances in which patients had bone marrow biopsies that were positive for dense infiltrates but they had normal tryptases, normal receptor expression, no D816V mutation, and no spindled mast cells.  So what do you have then?  Beats me.  Of these two patients, one has a diagnosis of MCAS and the other has a diagnosis of MMAS. (And if we're being picky, neither of them have known systemic reactions to bee stings.)  My guess is that if they had repeat biopsies, they would eventually find spindled cells and CD2/CD25 expression.  I think they are both probably very early in their diseases and we just caught it while it was developing.  

"Bone pain aggressive mastocytosis"

Aggressive systemic mastocytosis is defined as systemic mastocytosis with at least one C finding: low blood cell count; impaired liver function; free fluid in the abdomen; high pressure in the vessels connecting the liver and digestive organs; large osteolytic lesions and/or pathological fractures; impaired splenic function; malabsorption with weight loss; low serum albumin. 

Mast cells are involved in bone loss and osteolysis and/or fractures causes by osteolysis are not uncommon in ASM.  Mast cells release factors associated with bone resorption, bone formation and bone remodeling.  Having more mast cells speeds up these processes. 

Conventional wisdom has long held that bone pain in mastocytosis patients came from inside the bone, where too many mast cells inhabited the marrow.  However, consistent reports of bone pain from patients with non-proliferative disease (MCAS) indicate that this may not be true.  In particular, histamine is known to be irritating to osteocytes (bone cells) and may be causing pain from outside the bones. 

"Effects of systemic mast cell disease"

This is more loaded than the other topics because it is so wide reaching.  Regarding symptoms, it can cause low blood pressure, rapid heart beat, flushing, rashes, hives, itching, abdominal pain, nausea, vomiting, diarrhea (or constipation), numbness, tingling, headaches, sinus pain, swelling, coughing, difficulty breathing and more.  Regarding long term damage, mast cell disease can cause organ damage due both to mast cell infiltration and the effects of anaphylaxis and mast cell mediators.  Regarding lifestyle and such, mast cell disease can restrict your activities, your ability to work and affect your relationships with others.  I have dozens of entries in this blog regarding these topics; if you have specific questions, please let me know.

"How to activate mast cells"

If you have mast cell disease, unfortunately, activating mast cells is not hard.  Common triggers include sunlight, heat, pressure, exercise.  stress.  Common food triggers include soy, alcohol, vinegar, tomatoes, and like a million other things.  I use the low histamine diet described by the Canadian Mastocytosis Society and have found it to be pretty accurate for me.  That said, everyone is different and triggers are super individual.  I just blew my nose too hard and started degranulating.  Activating mast cells is not so hard.

"Mast cell activation disorder prognosis"

Every reliable source reports that patients with mast cell activation disorder have normal life expectancies.  The data looks good to me.  If you think I’m a reliable source, that’s also what I’m reporting. 

"Mast cell and high heart rate"

Tachycardia (heartbeat over 110 bpm) is a common symptom for mast cell patients.  When mast cells degranulate, the histamine released causes blood vessels to leak fluid into the tissues and out of the blood stream.  This lowers the blood pressure.   The body responds with “reflex tachycardia.”  This means that your heart starts beating faster in response to the drop in blood pressure.

Many mast cell patients also have POTS, which causes tachycardia when moving to a standing position.  My POTS is managed effectively with fluid infusions and a conscious effort to not stand up quickly.

I have a PICC line and about a month after I had it placed, I started having really bad tachycardia at night.  I mentioned it to my infusion nurse who told me that this can be a sign that the end of the PICC line is too close to a heart valve, which can increase heart rate in response.  She pulled my line out about a centimeter and the problem went away.  Not exactly mast cell disease related but I know a lot of us have central lines so I figured it was worth mentioning.