Authors and Disclosures
Glenn T. Furuta

Digestive Health Institute, Section of Pediatric Gastroenterology, Hepatology and Nutrition, Gastrointestinal Eosinophilic Diseases Program, The Children's Hospital, National Jewish Health, Mucosal Inflammation Program, Department of Pediatrics, University of Colorado Denver School of Medicine, Aurora, Colorado, USA

Correspondence to
Glenn T. Furuta, 13123 East 16th Ave., B290 Aurora, CO 80045, USA Tel: +1 727 777 7457; fax: +1 720 777 7277; e-mail: Furuta.Glenn@tchden.org

Needing some input....

Ok... so Brie tested negative for Mastocytosis and Monoclonal MCAS which is wonderful news in itself.  We are left with a a diagnosis of MCAS.

But I have some unanswered questions that I can't make heads or tails of.  I just received some of her records from Boston and I do not know what the results mean....... or even if they are significant at all.

The bone marrow biopsy results state "The bone marrow was slightly hypocellular for age with rare mast cells by stains / immunohistochemistry, not consistent with systemic mastocytosis.  PCR from bone marrow aspirate and peripheral blood were negative for the Kit Asp816Val mutation. A small number of mast cells were identified in the bone marrow aspirate, a subset which showed expression of CD25, without CD2 co-expression."

From what I can understand from personal research is CD25 can be related to autoimmune, leukemia / lymphoma, or mastocytosis - but apparently there wasn't enough of this for any diagnosis or concern (but should this be monitored for an increase down the road) Should it be present at all??? And what does it mean when they say "without CD2 co-expression"??

Thanks for any advice, insight or help you can offer me here.

                                                                     Kim

Lisa;

Your response was helpful as you said perfectly exactly what my thoughts were.  I just wasn't sure if how I interpreted it was accurate and hearing my thoughts in your words tells me what I need as you have always been a tremendous and accurate source of medical information for me.

Dr. Castell's asked me to set up a phone consult for 3-4 weeks after our trip to Boston but when I called to set this up they said she had left the country and won't be back until August 23rd so I have a long wait and anxiously wanted some input prior to that time so I decided to see what all of you thought.

                                                  Thank You!  Kim

Hey Kim!!!

Things are nice and quiet with you!!!   How nice!!!  It must mean that Brie is doing better!!

How's she doing and HOW ARE YOU???


LISA

Well, I´m REALLY sorry to hear how things are going, Kim!!  I was hoping that no news was good news!!!   I can´t imagine the emotional stress it´s causing you and your family.  It´s hard enough having your sister in trouble but to have your mother as well and a niece on top of it!! YIKES!!

Listen, Kim, as to your Mom and sister, I don´t know how close Brie is to your sister, but I have NO DOUBTS that she´s close to your Mom.  Kim, if you are feeling stressed, just imagine that going on in Brie and multiply it by 100 and this is what you will get if and when your mom passes away.  I´m not saying anything about that happening any time soon and I certainly hope it doesn´t, but if it should you´ve got to know ahead of time and PREPARE for what can happen.  

Kim, our emotions can create real havoc within us. I know you´ve seen Brie go through temper tantrums, but crying can create problems too!  I was amazed when I heard some bad news a little while ago and it got me doing some heavy crying and low and behold I found myself right on the edge of anaphyalxis!!  I hadn´t realized that crying and the emotions that would provoke it could push us over the edge, but it taught me that it´s serious business.  

If your Mom is having serious problems and is in serious danger, Kim, then, you need to prepare for the worst so that when Brie gets the news, you´ve got some back up meds to help deal with the triggering it´s going to cause.  Ask your doctor for some kind of sedative which will not knock her out, but only take the edge off of her emotions, especially in the beginning right after she gets hit with the news.  You already will have your hands plenty full dealing with your own feelings and having your other two kids to also have to consider the very last thing you need is to be rushing Brie to the hospital on top of it all!!    So, consider my advice here, for her sake as well as your own, to have some pediatric strength meds just in case.  I´m praying you won´t need them for some time way distant in the future, but you should have something to help keep her calm in case this happens.  It´s just preventative medicine against the anaphylaxis it can cuase her.  

I´m sorry to hear about the adrenal crisis!  This is another reason why you need to be prepared if your Mom should pass, for all of this is going to go haywire with Brie´s emotional response.  You should also let your doctor know of what is happening so that he´s also aware of how to respond.

Yes, Kim, we do and can get sick really quickly!!  This is part of what leaves us patients on edge, for we can be perfectly fine one moment and the next our world turns upside down!  It´s hard to feel secure this way!!!   But thankfully, you´ve gained so much understanding and your very hard work has finally payed off!!   YOU´VE GOT ANSWERS and this is PERFECT!!!

So, I´m sorry things are not well with you all.  Again, I´ll keep you in my prayers and hope that everything improves quickly!!!

Hugs to you!!!!

Lisa

Have you asked Dr. Castells about this?   I'll bet you anything it's connected for the little bit I just read does say that it's been associated with other neoplastic diseases.  If you will note, they state that it's mainly been associated with AML.  Well, the aggressive form of masto, which is very, very rare, is indeed found with myeloproliferative leukemia and since the mast cell is a cell which comes from the myeloproliferative chain of blood cells, I'll bet that there is a specific genetic defect which reveals this and is connected to it, Kim.  Yet, because they've not pinpointed the genetic defects for either disease yet....you go with only having guesses to go upon!  So, I'll bet you've got a piece to the bigger puzzle in Brie's case, YES!



Neutrophilic eccrine hidradenitis (NEH) was initially described in acute myelogenous leukemia (AML) patients undergoing chemotherapy.[1] Neutrophilic eccrine hidradenitis has since been reported in persons with various neoplastic and nonneoplastic conditions and in otherwise healthy individuals; however, most documented cases have continued to be observed in the setting of AML, usually in association with chemotherapy;


http://emedicine.medscape.com/article/1070937-overview

Hi Kim,
So good to see you on the forum.  I have been thinking about you and Brie.  Rylee knows that Brie and Jarrod (Judy's son who is 12 yrs. old) all share similar issues.  I received a message from Judy the other day and she said to tell Rylee that Jarrod said hello.  That made Rylee smile.  Please let Brie know that although Rylee has not met her, that she speaks of her and wonders how she is doing.  
Is Brie currently able to attend school?  Rylee went to school last Monday (9/25) but then was so sick she could not attend the rest of the week.  However, she was able to go yesterday and so far today she is still at school.  She was sooo excited when she woke up this morning that she was feeling so good.  I am hopeful that all the H1, H2 blockers, Gastrocrom, and Prednisone are beginning to stabilize her condition.  Maybe I am speaking too soon but 2 days in a row of feeling good is more than we have experienced the last 6 months.  
I know that I have prayer warriors on this forum and that certainly makes a difference.  I am so sorry to hear you are going through so much with family tragedies.  Know that you are being prayed for and thought of!!  Give Brie a big hug from us and stay in touch as you can.  Take care......Angie

It has been soooo long since I have been on this site... not by choice.  Just other events going on in life.  I really miss reading all of the new posts from all of you.  Just wanted to say that Brie is doing "ok" at this time.  She continues to have spurts of doing well.... and then other times where she still struggles.  She has again had daily symptoms for the last month... but still much better then it used to be.  She continues to grow as a sweet individual each and every day. Her strength amazes me!!!! And she knows so much about her disease for her age that I am always impressed.  And I also know that this comes only with thanks to all of you!  I hope you all had wonderful Holidays and wish only the best for each of you in the New Year!!