Atypical CML / MPN: Hi, I was diagnosed with... - Leukaemia CARE

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Atypical CML / MPN

Acml
Acml
8 Replies

Hi, I was diagnosed with Atypical Chronic Myeloid Leukemia (aCML) in September 2016 (WBC 37,000 on 8/23/16) - classed as an MPN. ("Looks" like CML, but no Philadelphia chromosome.) High neutrophils, thrombocytopenia (platelets have drifted down from roughly 130-140 in Sept. to 96 and a few 80's in April-May 2017), mild anemia (marginally low hemoglobin, hematocrit). Apparently quite rare, so hard to find research on or other patients, but hope to find patients or information here. Night sweats (actually, for over 10 yrs. PCP was negligent/lazy about investigating thoroughly, despite my repeated complaints); somr torso itching; some weight loss (172 down to 162); now some mild spleen pain since about 3 weeks (as of 5/17/17). WBC has fluctuated between low 30's and mid 50's (57 was following 2 week course of supplements from naturopath: agaricus blazei, vascustatin, curcumin, vit. C, green tea pills, ddan shen, andrographis, vit. D. Supplements only made blood work worse. WBC and neutrophils increased (neuts to 90%), platelets a bit better, to 126, but scary result over all. High doses of all supplements (e.g. 36 pills of agaricus, etc.) Watching/waiting under heme/oncologist supervision every 6-8 weeks. On deck fot possible stem cell transplant (but acml so rare, no 10/10 matches in 10million+ donor national database; and very hesitant about transplant, and heme/onc conservative about proceding to. Realistic about graft-vs-host reaction and risk/continuing pain, as well as initial mortality risk from initial heavy chemo. Age 69yrs+9mos.

8 Replies
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AJA1717

I was diagnosed with CML in 2000. I have no Philadelphia chromosome, just the cryptic translocation of DNA from chromosome 22 to 9. No match for a transplant. Night sweats lasted about 2 years. Started taking imatinib in 2001 and have been well ever since.

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Marie_14
Marie_14
in reply to AJA1717

Gosh I can't believe there are two people and the wife of a patient on here! I never ever got to contact anyone for my husband apart from medical people. Most if them were excellent. One was diabolical. Good luck to you. Are you in UK or other country?

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Marie_14

My husband was diagnosed with the same thing in late 2013. After he was given hydroxyurea we asked after about 11 months for a second opinion. He was then put on Jakavi. He was told he was a guinea pig as they hadn't used it forbthis condition before. Apparently it is so rare a doctor can go their entire career and never see a case. There can be severe side effects with Jakavi in some cases. My husband died in February of a rare neurodegenerative disease but there was concern at one stage it might be the side effects of Jakavi.

I have seen a lot of positive comments about Imatnib also about Jakavi but you need to read up on the severe side effects rather than the normal nausea and so on.

What medication are you on now and where do you live?

I managed to get to talk to someone about where he should go for a second opinion and what doctor he should see.

Like you we couldn't find people who had this condition.

You need to get the best advice out there.

Good luck to you and if I can help please contact me.

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Acml

Thank you all for responses. Sorry for delay (lost my password, as usual). I am not in treatment yet - been watching and waiting. Hydroxurea would be first symptom (palliative) treatment; then stem cell transplant if progression accelerates. Been trying everything I can to find any legitimate natural foods/herbs/supplements that might actually limit or retard progression. So much of this stuff seems really questionable and seems to do nothing, but still hoping and trying, with accompanying skepticism, as I would like to find anything other then chemo before having to go to that. If anyone has had any genuine success with anything, I would appreciate knowing about it. I am not in UK; in Boston, MA. Major hospitals have confirmed aCML as most likely diagnosis.

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Gerowgiraud

Hello -- Don't know if you still are involved in your message 2 years ago. I'd just say, if you are, I'd seek a second opinion if I had your readings and somebody told me i had aCML. You have a very high platelet count. My platelet count went down to 9 recently, and I need platelet transfusions about every 8 or 9 days. This raises my platelet count up into the 30s and then it comes right back down. My WBC is presently at 80. My Hemoglobin is at 9.2 but it has got to below 8 at which time I need a blood transfusion. When I read you had aCML I just wondered if your diagnosis is right, and especially to consider a bone marrow transplant with those readings? Like I said, I'd seek a second opinion. Good luck, in any case.

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rumshiba

Hi, I've been diagnosed with aCML at end of 2017, when my normal WBC count usually at 5,000 or so shot up to 10,000, and when I had an angiogram in early May 2018, my WBC shot up to 25,000 at which time a hematologist at the hospital gave me a bone marrow biopsy. At first she said it was CML and gave me literature on different target therapy drugs which I would be using shortly. Then at the next appointment,she told me I don't have CML because of no BRC-ABl nor the Phil.chromosome. I decided on a second opinion for my bladder cancer and the new hospital did wonders on that and it was resolved. Back to my aCML, the new hematologist gave me a third test to check whether I had the aberrant gene for CML and it was confirmed I don't. Also my neutrophils are high, my platelet is just below minimum normal, sometimes passing as normal, but usually a little low. March 2019, my WBC count is up to 63,000, but my RBC are still a little over 10. My LDH is sky high at 650. However, I don't have night sweats and basically I'm still fine, except I get tired easily. At the age of 77, getting more tired easily doesn't ring an alarm bell. I'm enrolled for clinical trials and am having molecular profiling done to find all my mutations and see if any drug can address them. I have one big question to ask: when we read all the literature on aCML, it always says 'poor prognosis', with overall survival at 25 months or so. How long does one live with aCML, citing a specific case? Has anyone lived 5 years with aCML? There is hardly any data available because it is such a rare disease. I asked my hematologist how high my WBC count would have to be to start internal bleeding, and he said aroun 200,000. So I told him I still have two-thirds to go. Hopefully, he will find some drugs, especially immunotherapy, to try on my mutations. Would appreciate any comments, thanks. Rumshiba

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Dee5588

Hi, my nan had just been diagnosed with aCML I'm just looking for any advice, help anything. Stories of people who have had this please?

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Gerowgiraud

Hi -- I was diagnosed with aCML on October 29th this year. This was a corrected diagnosis of CMML (Chronic Myelomonocytic Leukemia which was diagnosed on January 31, 2017. This is a reason why it is best to have a leukemia specialist do the testing. I take it your Nan's diagnosis came after a bone-marrow scan. There are other tests they can do to determine treatment targeting options. If you don't have a leukemia specialist to help you handle this, try to see one. It is worthwhile to see one even if you have to travel some distance to do so. Just say you think it might be a good idea to seek a second opinion and your doctor should agree. Hematologists and cancer specialists are good, but they are unlikely to have the specific experience that would help make the best recommendations necessary. Also, the laboratories they deal with may not be as sophisticated as those a leukemia specialist uses so it is possible to make a mistaken diagnosis in the lab testikng. Below are some links which discuss some treatments that they have used to treat aCML, in some cases successfully. Be aware, however, that each individual case needs to be examined specifically. For example, none of these treatments would work for me and if they were used for me could make my condition worse. -- David

ncbi.nlm.nih.gov/pubmed/300...

ascopubs.org/doi/full/10.12...

futuremedicine.com/doi/10.2...

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