I am waiting for results of my blood test!!

I got the results of my bone marrow test in April.. and the Doc says they will do a blood test in three months ....so I went in this week and gave some blood for testing..

What are they looking for in the blood.. what will be the bad news...what is the main thing... is it Tryptase levels... some times I am in shock so don't seem to know what to ask the Doc..

I read that if the mast cells go into the bone marrow your not going to live very long...so what are the Doc looking for!!!  they found mast cells in the bone marrow...Ellen

Ellen,

Mast cells are supposed to be in the bone marrow.  It becomes a problem when there are too many mast cells and/or if they are mutated.  Some people have too many mast cells, but they're proliferating very slowly, and those people live a normal life span.  Symptoms occur because there are too many cells and, when they degranulate, they dump a lot of mediators, including histamine, prostaglandins, heparin, serotonin, and/or others.  Those can cause mild symptoms or anaphylaxis.

When Aggressive Systemic Mastocytosis or Mast Cell Leukemia are diagnosed, and they're extremely rare, then some sort of chemotherapy is usually prescribed.  It usually progresses very quickly, and treatment is started also quickly.  Some of the newer drugs are reversing symptoms in some ASM patients.  Be careful about reading older articles online.  Thinking about mast cell diseases and their treatment has changed dramatically even in the last 5 years.

There are many things that are tested routinely in MC disorder patients, including tryptase level.  If you're worried, call the doctor's office and ask the nurse what the doctor believes your diagnose to be and for the results of your blood test.

It's hard to remember what to ask the doctor, so begin now making a list of questions you can take with you to your next appointment.

Ellen, first of all, calm down.  If they told you you have Systemic Mastocytosis, Many doctors still consider Systemic Mastocytosis a form of Leukemia but its not The Mast Cell Leukemia.  When I was going to MD Anderson in 2004/2005 and in the Gleevec trials (chemo, I have SM) my oncologist said SM was a form of leukemia.  Trust me if you had Mast Cell Leukemia there would be no need for blood test in 3 months you would be nearly dead by then.  More than likely you are stuck with Systemic Mastocytosis doesn't mean your going to die anytime soon.  Sometimes you may feel so bad you wish you would, I go thru that alot, but doesn't mean your going to.  They will probably check your blood work every few months CBC etc to see if its causing anemia or other problems.  Do you have a copy of your bone marrow biopsy?  If you do, how many or what percentage of mast cells were in the bone marrow?  Did they describe the shape of the mast cells, spindle, etc.?  Did they stain positive cd117, cd2 and cd25?   That is what my mast cells are and I am still here and mine turned SM between 2000 and 2004 and I was originally diagnosed with UP in 1994.   So don't go into a depression about dying.  I am still here and lots of others are too.  You need to learn all you can about this disease, don't give up, that is the worst thing you can do.

Wendy

Joan,

Maybe someone else can clarify this too, but from what I know Mast Cells are NOT suppose to be in your bone marrow.  They are made there but they are immature and called something else and then travel thru your system and the Stem Cell tells it to turn into a mast cell.  If they are found as mature mast cells in the bone marrow then you have Systemic Mastocytosis.  Then they have to figure out if you have the mutation, if the mast cells are normal or atypical mutated.  From everything I have learned that is the difference between SM and MCAS/MCAD.  

Joan and Deb,

Now I am confused.  I have always been told by my docs that its abnormal to have mast cells in the bone marrow, if you do its because you have SM.  Now look at the 2nd page where Dr. Litchenburg (ooops spelling probably wrong) the doc from NC states that it's abnormal to have mature mast cells in the bone marrow.  The very first bone marrow biopsy I had done in 1999 there were no mast cells in my bone marrow.  So there fore I had not progressed to SM but in 2004 there were many.  
I don't mean to be confrontational but we need to get the correct info out there for people.

Okay, before we all start splitting hairs here, there are some things we need to remember.   There is a difference between immature and mature MCs and there is a difference between clonal and healthy MCs.  When we are talking about SM, we need to keep this in mind.  

When the authorities are talking about SM they are not talking only about individual mast cells, they are also talking about AGGREGATES of ABNORMAL MCs.  They are also talking about SPINDLE SHAPED MCs.  REGARDLESS of whether the MCs are isolated round cells, or focals of MC aggregates or that of spindle shaped MCs, they all must be tested either through flow cytometry or through immunohistochemical testing.  They must test POSITIVE for CD2 and/or CD25 as well as other markers.  If the aggregates or other MCs are not at least 15 to the field, then this is not yet to the point of systemic mastocytosis.  These are the WHO requirements for SM.  

Now, in going back to Dr. Lichtenberg's comments:  

"Part of what makes any call mature(red blood cell, white blood cell, Mast cell) is the communication it gets from the surrounding environment. When mast cells are developing, they mature in tissues at sites that they are programed to go to, sites of wound healing, and sites of allergic inflammation. When Mast cells mature in the bone marrow, it is because the cell thinks it is getting a signal telling it to become a mast cell. Many people with Mastocytosis have a mutation in KIT, which causes this errant signal. So finding mast cells in the bone marrow is abnormal, and is criteria for mastocytosis."


I'm not about to question what Dr, Lichtenberg has to say for he's fresh from the NIH and Dr. Metcalfe's tutelage and I'm sure he knows his stuff.  So, if Dr. Lichtenberg is saying that mature MCs within the marrow are part of the criteria for mastocytosis, then this must be so, and perhaps he can help us gain more understanding as to what he is saying here.

What I do know, however, is that when the doctors do find mature MCs within the marrow, they have testing that they do in order to determine what condition that MC is in for there is also what is called an MC hyperplasia which can happen within the marrow and this is not mastocytosis.  An MC hyperplasia is an over abundance of normal, healthy MCs.  Why this is I'm not sure, but I know that they do not consider this as mastocytosis, it is another entity.  

This is why the WHO Consensus document has very specific criteria for what exactly Systemic Mastocytosis is and having a few loose and even mature MCs wandering about is not something where you've got to be overly concerned.  It's abnormal, yes, but it's not dangerous.   And for doctors to be coming out and saying that you have mast cell leukemia is really rather wreckless for this is a very seriously situation.   According to the WHO Criteria you must have over 20% of the marrow filled with the MC aggregates!   That's a lot of marrow!!!  Anything less and you can NOT call it MCL!  And it's neglegent to be calling mastocytosis a form of leukemia for it is NOT considered this by the authorities.  It has been recently reclassified as a myelodyplastic disorder, but this does not make it a leukemia!

This is what the WHO consensus document says on MCL:

Wright-Giemsa is the standard-stain for MC-detection
and enumeration in bone marrow smears [9,10,54]. It is
standard that MC are counted (percent of nucleated
cells) at considerable distance from marrow particles
[9,10,54].  If ≥ 20% of cells in bone marrow smears are MC, the diagnosis is MCL.  The morphology of MC is recorded using
published criteria for morphological grading . The non-MC-compartment should also be examined : Mild dysplasia is found in most patients with SM . If dysplasia is prominent, the patient
should be examined for additional signs of smouldering
SM (SSM) or an associated myelodysplastic syndrome (MDS). Other relevant findings include eosinophilia, basophilia, or an increase in blasts (see also SM-AHNMD).  



Now in reading a bit further in this document, in regards to the mature mast cells, this is what the document has to say:

Cell type  -  Mature
Criteria   -  mast cell round cell, round nucleus, well granulated
Clinical Significance  -  usually seen together with atypical mast cells type I in patients with ISM; if most cells are round and mature, consider the rare sub-variant ‘well differentiated SM’ (± KIT F522C or other KIT mutations)

The burden of neoplastic MC (and serum tryptase) remains
remarkably stable over years in most patients with ISM
(even in most with SSM). In these patients, no repeated
BME is required. However, a BME is warranted if there
is evidence of disease progression (e.g. increase in serum
tryptase).  The most important follow up parameter is tryptase
IHC to evaluate MC-infiltration.


So, I think that if we could ask either for a comment from Dr. Lichtenberg or from perhaps another authority like Dr. Akin who could give us a  clarification, this would be extremely helpful.



Now, before I close, I'd like to say one more thing to you, Ellen.   I hate to say this but you have fallen into the same trap that we all fall into in the beginning and that we all must fight against repeating.   Ellen, I did this when the suspicion for the Carcinoid Syndrome was raised in my case.   Masto and the Carcinoid tumor have the same syndrome of symptoms with the flush being the major telltale symptom.  Yet of the two diseases, the Carcinoid is the more deadly by far!  I was PETRIFIED that I had the tumor and it IS CANCER!  I had convinced myself based upon my symptoms and what I had read that I had the tumor to the point I almost didn't believe my doctor when he said he was 99% sure I didn't have it!!  My exams were all negative for the carcinoid, but the problem was that he had never heard of anybody possibly having anaphylaxis with hypertension and so this is what gave him the 1% doubt as to my not having the tumor.  The doubt was important enough, however, that it forced us to go to the highest authorities in Brazil on these tumors.  My doctor was perhaps #3 in Brazil and he sent me on to the only doctors higher than him.  They confirmed his conviction that I didn't have the tumor, but they also didn't know masto and it wasn't until an autoimmune marker showed up positive that we knew it was masto.  But it was a very hard lesson to learn that I was so very, very wrong and that I had fallen into the typical trap that patients who have access to the internet fall into!!    

This is why doctors are not too thrilled with the internet for patients don't have the training a doctor has.  They are taught to not trust their feeling but to DEMAND EVIDENCE and rely upon EXPERIENCE to tell them how a patient is doing.   Yet, when doctors are dealing with as rare a disease as masto, they make mistakes too!!   These highest authority oncologists ended up giving me a diagnosis of Hysteria!!!  This is because they could not believe that anybody could go through anaphylaxis with pressure of 220/110 following contrast use!!!  They couldn't believe what I described to be syncope to be syncope and since they'd never heard of syncope lasting for 3 full hours, I must have been out of my mind instead!!  This is why they diagnosed me with Hysteria!!   They knew nothing about masto!!!!!!!!

We have long since established my case as indeed MCAS and possibly MMAS and it was Dr. Castells herself who confirmed this diagnosis.  These doctors were horribly WRONG and extremely neglegent for they didn't have the authority to do anything more than RULE OUT THE CARCINOID, which they did.   They misjudged my fears and felt that my anxiety had everything to do in producing psychosomatic symptoms!

This is why I say to you now, Ellen, that although you may be working with excellent doctors, they may not be very experienced with mastocytosis and may not have the slightest clue of what they are doing.  One of my doctors, an immunologist who specialized in rare diseases had treated only 3 patients in his over 20 year career!  Think about it - the man specializes in rare diseases and had only 3 patients to treat and he had never diagnosed a single one of them!!   I'll bet you anything your doctors really don't know mastocytosisa!   You need to go to a specialist or an authority in this disease for it's very difficult to diagnose and many patients have been wrongly diagnosed.  This would explain their telling you it's a type of leukemia!

So, please, don't jump to any conclusions on your own and don't settle for final the statements of doctors who are not specialised or experts in this disease.  It's extremely difficult to diagnose and you really need doctors who know what they are doing!

I hope this helps!

Lisa

EXACTLY!!!  Thanks loads Peter, I´d forgotten to put that info.  

So, Ellen, you may be dealing with some major issues with your bone marrow and you may have another blood disorder connected to this and this is why you are needing to see some high authorities QUICKLY.   You must run after the opinion of other doctors and not rely solely upon your oncologists opinion.    

Ellen, one of the reasons why we depend so much on Joan´s opinion is because she´s got smoldering masto and is both Dr. Gotlib´s and Dr. Akin´s patient and she´s had to be monitored closely, so she´s done some study in this.  We also have some aggressive masto patients here who will know much more about this area than most of us do since we don´t have to concern ourselves too much with this.  They can help you if you´re needing it, but I think they will ALL tell you, you must not depend solely upon your own doctors.   They will tell you that your doctors can´t make this call.  And the fact your doctor isn´t wanting Dr. Akin´s opinion to me is very suspicious!  I personally would not trust my doctor if he said this to me for Dr. Akin is a recognized authority and SM is his area of specialty.   It seems rather arrogant if you are asking me and since this is so very serious of a situation you are facing, you are only wise to question your doctor´s capacity to handle this and require the involvement of an authority.

One of our members comes to mind.  She´s facing a bone marrow transplant!   This has been done so very rarely that the doctors don´t really have much to go on in the literature!!  This patient, when her doctors said TRANSPLANT wrote to Dr. Akin herself and he said COME and she hopped the next plane to Boston.   Dr. Akin went over her and said that he would gladly help support her doctor, which he WANTED.  Dr. Akin sent her to see one of the masto hematologists at Harvard and as THREE doctors putting their heads together, this member´s oncologist, together with Dr. Akin and his hematologist ANALYZED her condition together and are MONITORING her.  She is not yet facing this transplant for the doctors are very closely monitoring her platelets.  She´s already been through chemo and didn´t respond well enough and is still in danger thus the consideration for a BM transplant, but this is such a high risk situation that it could very well kill her and so THESE THREE DOCTORS are WORKING TOGETHER for they are recognizing the peril that this member is facing.  

So, this is why I think that your doctor, not wanting to work with Dr. Akin for me is HUGE WARNING SIGNS that something is not right, that he´s clearly not recognizing the situation.  And this makes me feel more in peace about your situation for this indicates that he´s not understanding mastocytosis and your situation is probably not as dire as you are thinking it is.  

Regardless, however, I would STRONGLY urge you to ask Deborah for Dr. Akin´s email address and that you write to him YOURSELF.  Ask him what he thinks of your test results and whether you are in need of seeing him ASAP.   If he thinks you are in danger, he will say so.


I hope this helps!!!


Lisa

Joan what I read about Mast Cell leukemia is that if more than 20% of your bone marrow is atypical mast cells AND more than 10% of your circulating  blood is mast cells then its considered MCL.  I have only met 3 people with this.  One I kept in touch with for a while.  If I remember correctly he lived almost a year with MCL before he passed away.  He was only in his 50's.  He started with UP years before and it slowly progressed to the MCL.