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Atypical CML - newly diagnosed


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#1 CheriJ

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Posted 25 July 2012 - 08:03 PM

Hi

My name is Cheri and I was diagnosed about 3 weeks ago with atypical CML.  I've been doing a lot of reading and am pretty terrified at this point because it appears the TKI's don't work with my type of CML.  Is there anyone here that has acml and would be willing to give me some feedback on what to expect treatment wise and otherwise?

I will be working with MD Anderson here in Arizona., am currently waiting assignment of a Doc.  Of course right after I was diagnosed there was an insurance change... story of my life.  I feel pretty blessed though as MDA is about a 1/2 mile from my house. 

I keep reading the life expectancy is around 24 months.  I'm scared and feeling very alone.

Thanks.



#2 scuba

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Posted 25 July 2012 - 09:25 PM

Hi Cheri - I assume you are talking about the Banner MD Anderson Center in Phoenix - correct?

You will be in good hands at that location.

Also - there will be a lot of people on this forum who will introduce themselves to you and share with you lots of information. You're not alone. Some of it may seem overwhelming at first, but it all sorts itself out.

I do recommend you read Trey's blog (you'll meet Trey here soon enough - best to let him introduce himself. He's kind of famous here):

http://treyscml.blogspot.com/

His blog is a very good summary of what you will be dealing with and sorts out the terminology. Think of it as a reading assignment.

Can you tell us what CML you have? (breakpoints, etc.) - Why is it atypical?

And finally - you will survive. CML is beatable - or at least manageable. There are lots of trials for new drugs as well as novel uses of the current ones. You have a long life ahead. As I am fond of saying - you will die of something else. Not CML.

Welcome to the group

Michael


Diagnosed 11 May 2011 (100% FiSH, 155% PCR)

with b2a2 BCR-ABL fusion transcript coding for the 210kDa BCR-ABL protein

 

Sprycel: 20 mg per day - taken at lights out with Quercetin and/or Magnesium Taurate

6-8 grams Curcumin C3 complex.

 

2015 PCR: < 0.01% (M.D. Anderson scale)

2016 PCR: < 0.01% (M.D. Anderson scale) 

March        2017 PCR:     0.01% (M.D. Anderson scale)

June          2017 PCR:     "undetected"

September 2017 PCR:     "undetected"


#3 Trey

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Posted 25 July 2012 - 09:54 PM

Cheri,

To help others here understand, Atypical CML is:
"...a chronic myeloproliferative disorder with a clinical and hematological picture similar to chronic myelogenous leukemia (CML) but lacking the Philadelphia Chromosome and BCR-ABL rearrangement....."

http://atlasgenetics...aCMLID2117.html

So you are negative for the Philadelphia Chromosome and have no indication of bcr-abl, but your blood counts look like CML (high WBC and maybe high platelets).  This is a very rare form of CML, and there is some debate about whether it is actually CML leukemia, or maybe closer to CMML leukemia, or maybe some separate form of a myeloproliferative disease entirely. 

A few questions so we can  possibly help you:

What are the signs of your aCML (WBC, platelets, blast count, other features)?

What did the BMB report say?

Did you have a JAK2 mutation test?

What is the proposed treatment plan so far (I realize you have not yet been to MDA)?


Here is some info on the subject of  aCML:
http://www.pubmedcen...i?artid=2082654

http://www.seminonco...0293-4/fulltext



#4 Susan61

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Posted 25 July 2012 - 10:23 PM

Hi Cheri:  Unfortunately I know nothing about aTypical CML, but we do have some knowledgeable people here who can help you out.  I can only pray that you get some real answers when you see the doctor at MD Anderson.  Please keep us informed as we will all support you in whatever you have to do.  We have a great group here, and we listen and try to help whenever we can.  Glad you posted to us, and wait till you get all your test results in before you think the  worst.

God Bless You

Susan



#5 Lori's okay

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Posted 25 July 2012 - 10:47 PM

Hi Cheri,

I sure hope you get some more encouraging news when your doctor is assigned.  Do remember as you do research and reading online that some of the info out there is outdated.  There is SO much research being done in this area, perhaps there will be a clinical trial for you?  Anyway, it is great you live so close to a very good cancer center.

I will be in the Phoenix area in September for a week.  Even though these diseases sound pretty dissimilar I'd be happy to talk.  Please know you are in my thoughts. 

Hugs coming your way,

Lori


DX 09-2011 PCR 8.08 not IS WBC 17 , Began Tasigna 600mg  

in 2012 Tasigna 450mg/day ,in 2013-2017 Tasigna 300mg/day

DATE/PCR

09-11/ 8.08 not IS

03-12/ 0.054 not IS

06-12/ 0.035 not IS

09-12/ PCRU, 01-13/ PCRU

4-13/ 0.042 IS

7-13/ 0.014 IS

11-13/ PCRU, 04-14/ PCRU

8-14/ 0.006 IS

PCRU: 12-14/ 05-15/ 10-15/ 02-16/ 

09-16/ 02-17/ 09-17/ 

10-17 tapered off 

11-3-17 Stopped Tasigna

1-15-18 still PCRU

 


#6 Happycat

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Posted 26 July 2012 - 04:45 AM

Cheri,

I can understand why you must feel scared and all alone. The aCML is rare indeed. I'm glad you are going to MDA. I know you are waiting for a doc assignment there. Are they trying to find you an aCML specialist?  Not sure if they exist, since it is fairly rare. Anyway, if it were me, I'd try to find such a specialist if possible.

As Trey said, pls let us know a little more about your dx if possible. Do they know for sure it is aCML?

All my best,

Traci



#7 scuba

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Posted 26 July 2012 - 06:41 AM

Trey - Thanks for the edification. I found the references you cited most helpful in explaining  aCML and also explaining myeloproliferative disorders in general. I know that MD Anderson in Houston is doing lots of research in this area. I assume that whoever Cheri is paired with in Phoenix will be able to tap the work in Houston.


Diagnosed 11 May 2011 (100% FiSH, 155% PCR)

with b2a2 BCR-ABL fusion transcript coding for the 210kDa BCR-ABL protein

 

Sprycel: 20 mg per day - taken at lights out with Quercetin and/or Magnesium Taurate

6-8 grams Curcumin C3 complex.

 

2015 PCR: < 0.01% (M.D. Anderson scale)

2016 PCR: < 0.01% (M.D. Anderson scale) 

March        2017 PCR:     0.01% (M.D. Anderson scale)

June          2017 PCR:     "undetected"

September 2017 PCR:     "undetected"


#8 CheriJ

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Posted 26 July 2012 - 09:31 PM

Thank you all for the responses and the encouraging words.   I have had a slightly elevated wbc for about 1 1/2 years and an enlarged spleen. Having a lot of back pain, did MRI which came back with decreased fatty conversion in bone marrow.    PCP sent me to a hematologist, had a BMB.  Hypercellular marrow, 90% hemopoetic hypercellularity.  Granulocytic and megakaryoctyic hyperplasia.  No blasts in blood or marrow. slightly elevated neutrophils. Baso =0, Mono =5

Fish and the PCR test came back without any abnormalities in the chromosomes.  I don't think I have been tested for Jak2? Is there a different test for that?  I asked the doc and he said no abnormalities.   In the middle of all this my WBC went back to normal.  So he wanted to do monthly testing to wait to start treatment until there was a change in my wbc and/or platelets.  And that's where I'm at. 

I'm still trying to figure out what all that means.  Thank you Trey for the links.  I will be reading them avidly in a few minutes.

Thanks again for all the responses, it's so nice to be able to talk about this.

Cheri



#9 Marnie

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Posted 26 July 2012 - 10:28 PM

HI, Cheri. . .

I don't have anything to add except my good wishes.  Fingers will be crossed for you as you meet with the docs at MD Anderson.

Marnie



#10 scuba

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Posted 27 July 2012 - 05:47 AM

Trey - Would there not be cytogenetic evidence for aCML? It almost seems to me that Cheri's hyperplasia may be a result and/or response to an infection or some other immune response. She writes that her blood counts have returned to normal. Is it possible there may be nothing wrong with her bone marrow - or some myeloproliferative disorder may be developing?

Just curious ...

http://aplasticcentr...w_disorders.htm

(article I was reading on general bone marrow disorders. It's a nice summary)


Diagnosed 11 May 2011 (100% FiSH, 155% PCR)

with b2a2 BCR-ABL fusion transcript coding for the 210kDa BCR-ABL protein

 

Sprycel: 20 mg per day - taken at lights out with Quercetin and/or Magnesium Taurate

6-8 grams Curcumin C3 complex.

 

2015 PCR: < 0.01% (M.D. Anderson scale)

2016 PCR: < 0.01% (M.D. Anderson scale) 

March        2017 PCR:     0.01% (M.D. Anderson scale)

June          2017 PCR:     "undetected"

September 2017 PCR:     "undetected"


#11 pammartin

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Posted 27 July 2012 - 09:00 AM

Hi Cheri,

I echo Marnie's words, I have nothing to add because I know little of your diagnosis, but wanted to say welcome to the group.  Best of luck in your upcoming appointments and please let us know how you are doing.

Pam



#12 Trey

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Posted 27 July 2012 - 09:30 AM

Cheri,

If this is aCML, it has been discovered very early.  I use the word "if" because aCML is difficult to diagnose and usually includes much higher WBC than you have shown, and high blast count (yours is zero).  So although I do not have all the information, it would seem difficult to properly diagnose this as aCML at this point.  I would hold onto a bit of scepticism about this until more testing is done. 

The JAK2 mutation test is a separate test.  You should ask the MDA folks if it has been done, and if they will do one.

From what you have said, your marrow cellularity is high (granulocytes and megakaryocytes -- which are pre-platelet cells), your spleen is enlarged, and your monocyte count is high.  The back pain is odd, and does not seem to be associated with aCML unless it is the spleen, which I doubt. 

With such symptoms and normal WBC, diagnosis is not easy.  It could even be very early stage regular CML that has not progressed enough yet to diagnose.  Or there are other possibilities.  My purpose is not to try to diagnose you, but simply to point out that I have a difficult time understanding why the docs would jump straight to a diagnosis of aCML at this point.  There is not a specific cytogenetic marker for aCML that would make a diagnosis easy to confirm (Scuba's question), and it is usually diagnosed by a lack of any other explanation.  Such a diagnosis does not seem possible at this point, and although it could be a lucky guess, you would prefer a better approach.

If you have other high or low counts or symptoms, they might provide more pieces to the puzzle. 



#13 mariebow

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Posted 27 July 2012 - 10:02 AM

Trey I sent you a pm a day a so or go. check your box.



#14 CheriJ

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Posted 27 July 2012 - 12:54 PM

Yes, I agree.  I am looking forward to a second opinion on this for sure.

I got the call this morning from BMDA.  Appointment on the 14th august.  Glad to be finally on my way one way or another.

My Doc will be Dr. Klueppelberg - has anyone heard of him?



#15 Trey

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Posted 27 July 2012 - 05:33 PM

I would want to see a true leukemia specialist.  His MDA resume does not show that.

Specializing in the following Diseases, Conditions, Procedures:
Multiple myeloma and other plasma cell disorders, lymphomas, myelodysplastic syndrome, brain cancers, head and neck cancers, thoracic cancers

http://www.bannerhea...tail.htm?id=532

You are not a typical case, so you need a great leukemia specialist to sort this out.  If it were me, I would ask for someone else or go somewhere else.



#16 Happycat

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Posted 28 July 2012 - 07:41 AM

Cheri,

I checked my "Neoplastic Hematopatholgy" textbook for aCML. It's hard to dx, so I'm not sure they've got it right. It might be a possible dx, but from what you've said, I would not say it is definitive. It could be some other myelodysplastic  syndrome, or something else altogether.

Major features:  WBC usually 30-50 x 10^9 in peripheral blood. Absolute monocytes high, %mono usually 3-10%. Lots of dysplasia seen in PB and marrow.  This is a distinguishing feature between aCML and CML.  Dysplasia  means lots of immature cells.  You mention hyperplasia (lots of cells, but should be mature).  Also, if mono high, could also be CMML. So still lots of questions on your dx. This is also complicated by the fact that your WBC went back to normal. I definitely would not buy aCML yet.

I will also say this. My PB initially showed lots of immature cells, promyelocytes in my case. I was originally dx'd with APML. Turns out the "dysplasia" was caused by a cortisone shot I'd had for neck pain. Cortisone causes a left shift in the blood, I think by simply shaking the maturing cell lines out of the marrow too early. My point is that some cases can have red herrings.  Yours could be one of those. I think you should (and will) have a complete re-evaluation at MDA.

Keep us posted,

Traci



#17 Happycat

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Posted 28 July 2012 - 07:43 AM

It might make sense if they looked at her records and suspect a myelodysplastic syndrome, rather than leukemia.

Traci



#18 CheriJ

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Posted 28 July 2012 - 12:07 PM

Thanks Traci - that's encouraging.  I know I don't fit the mold.  Prev doc went through all the other dx and I don't fit those either. So I hope they will run new bloodwork...  Maybe another BMB?  And see where I am at. 

I keep feeling that I don't really have leukemia.  My family says I am in denial.  Maybe.  But heck,  if there was something in black and white someone could show me then maybe I could be more accepting.  Course I bet everyone here went through that at one time or another.  I guess because I don't feel bad I don't want to believe there's anything wrong.  But then there's the BMB results...  ugh. 

Trey I am hoping that the doctors consult back and forth there.  My understanding is that you are assigned and you don't get a choice.  Is this incorrect? Do people ever get referred from one doc to another after being accepted into MDA?   Also I am wondering what kind of research he won his award for. I will do some searching to find out.

Thanks everyone for your thoughts and ideas.  In the end I think you all will be making a large contribution to helping me get the right kind of care.  I can't tell you how grateful I am for that and for the support.

Cheri



#19 PhoenixPat

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Posted 28 July 2012 - 11:53 PM

Hello Cheri- I'm a CML patient in Phoenix and am happy to connect with you offline as well as here.

You need to get your care at a facility that can treat you appropriately.

I was initially treated for CML at the Banner Bone Marrow Transplant Center. The Center was supposed to be part of the new Banner MD Anderson Cancer Center, but the medical group and MDA could not reach an agreement. As a result, the medical group split up. Some went to Mayo Clinic, some built a new transplant center at Scottsdale Healthcare. As a result of all this, Banner MD Anderson did not open with a strong blood cancers program. They are recruiting specialists now but they do NOT have a true CML specialist, even though they will tell you on the phone that they treat CML. Banner and the leukemia team in Houston do not work together at this time. Banner in the process of recruiting hematologists/oncologists, but they do not expect to even have a blood cancers treatment program really underway until this fall. I would strongly advise you to look elsewhere or to go to MDA Houston if you can.

.

The only true CML specialist in the Metro Phoenix area is Dr. Reuben Mesa at Mayo Clinic who specializes in myeloproliferative disorders and has the support of the main Mayo campus to tap into as needed. http://www.mayoclini...ography/SB00042

You may also want to check into specialists at the Arizona Cancer Center at the University of Arizona in Tucson. It is the only NCCN hospital based in Arizona. http://www.azcc.arizona.edu/ 

Again, I am in Phoenix and would be happy to talk with you on the phone or meet you for coffee. I would also like to make you aware of the Phoenix CML Connections group. You may not want to join right now, but I wanted to assure you that there are several of us here who will be happy to help you and share information and resources.

Warm regards,

Pat



#20 scuba

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Posted 29 July 2012 - 06:55 AM

Pat - Thanks for the clarification. I was told that Banner MD Anderson and the one in Houston collaborate. Banner and MD Anderson jointly created the center in September last year.

http://www.bannerhea...s/_About Us.htm.

But based on your experience - it seems Banner MD Anderson is not the place to go.

Cheri should seek care elsewhere - especially given that her case is not typical.


Diagnosed 11 May 2011 (100% FiSH, 155% PCR)

with b2a2 BCR-ABL fusion transcript coding for the 210kDa BCR-ABL protein

 

Sprycel: 20 mg per day - taken at lights out with Quercetin and/or Magnesium Taurate

6-8 grams Curcumin C3 complex.

 

2015 PCR: < 0.01% (M.D. Anderson scale)

2016 PCR: < 0.01% (M.D. Anderson scale) 

March        2017 PCR:     0.01% (M.D. Anderson scale)

June          2017 PCR:     "undetected"

September 2017 PCR:     "undetected"





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