Lisa
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Re: Bone marrow with mast cells.. what next??
Reply #10 - 06/15/12 at 17:17:12
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
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