Acute Myelogenous Leukemia (AML) Support Group
Acute myelogenous leukemia (AML), also known as acute myeloid leukemia, is a cancer of the myeloid line of blood cells. Patients with AML usually present with symptoms such as fatigue, bleeding, infection, prompting medical attention. An abnormal blood test reading will then result in further testing in a hospital with a hematologist to determine AML.
Hello team,
Just wanted to give a quick update.
I was supposed to be getting my first day of chemo today for BMT but the doctors at Stanford threw us a curve ball last week.
My NIH doctors had diagnosed me with Myeologenous Hypereosinophilic Syndrome/ Chronic Eosinophilic Leukemia (M-HES/CEL). This is a type of Myeloproliferative Neoplasm (MPN). After my third bone marrow biopsy, the doctors at Stanford had a slightly different approach.
Their diagnosis is Systemic Mastocytosis with an associated hematological non mast cell disease that is likely chronic eosinophilic leukemia (SM-AHNMD-CEL).
In addition to the vast number of eosinophils in my bone marrow, all of the biopsies have shown aggregates of spindle shaped mast cells. My NIH doc says that the differing diagnoses are semantics. Apparently eosinophils and mast cells hang in the same biological circles, so, in the absence of specific genetic clonality, it is impossible to say whether my disease is primarily eosinophilic with secondary mastocytosis or primarily mastocytosis with secondary eosinophilia.
Welcome to the wonderful world of having an uber rare disease. More than six months in and still no diagnosis. When I ask my docs for any sort of prognosis, they all look at each other and shrug their shoulders with the exception of saying that it will eventually kill me if we don't get it under control. That could be one month or five years.
Either way, we are looking at this new option as a blessing, despite the uncertainty. We are now establishing a path to getting this new drug (midostaurin, which is a TKI that has had limited success in AML patients).
The doctors did not want to leave me off of any treatment as my absolute eosinophil count (AEC) continues to slowly rise. Last CBC I had a WBC of 27 with AEC at 20.5. They started me on hydroxyurea (1 gram/day) to see if that would lower the counts as a bridging strategy to get to midostaurin.
Midostaurin just finished phase II trials and is not approved by the FDA. Novartis is the drug company and they must say yes for compassionate use as well as the FDA, which may take 2 months or more. Then another two months to see if it works. My doctor, who was primary investigator on the trial, is unsure if it will work but thinks it is worth the risk to wait to try. Of the 116 patients in the trial, 5 presented in a similar manner, (SM-AHNMD with CEL). 4 of those 5 had a complete resolution of their eosinophilia.
Additionally, for those of you following the Beat AML initiative, my doctor also sent off my blood for evaluation with Jeff Tyner using the same techniques for AML genome mapping with the hope that another TKI might be better suited for me than even midostaurin.
http://www.lls.org/who-we-are/a-new-approach-to-drug-discovery?src1=35591&src2=
As I have become more and more versed on this subject, I am more and more encouraged by the progress in beating myeologenous leukemias of all forms. I am fairly certain that in 20 years, leukemias of this variety will be treated first with induction, then TKI to vastly reduce chances of relapse and then BMT only in rare occasions. CML has mostly been beaten, replaced by taking a pill with few side effects. We can do this too with AML!
I would encourage all of you out there to write your docs to send any blood that may still be stored to Jeff Tyner and the lab if pathways exist. I remember Dave telling me at the beginning of this journey for me that we are all part of the process to beat this disease. I took that to heart and wholeheartedly agree.
What I would ask of the team for me personally is to continue praying, specifically for the wisdom to make the right choices between BMT and drug therapy, for protection of my heart and other organs from all the eosinophils, for hydroxyurea to work as a bridging strategy, and for midostaurin to be approved for me and to work.
If you have any experience with hydroxyurea or midostaurin, I would appreciate any info as well.
We are so thankful for you all and pray for everyone here daily. Hope and health for everyone.
Vr.
John
Just wanted to give a quick update.
I was supposed to be getting my first day of chemo today for BMT but the doctors at Stanford threw us a curve ball last week.
My NIH doctors had diagnosed me with Myeologenous Hypereosinophilic Syndrome/ Chronic Eosinophilic Leukemia (M-HES/CEL). This is a type of Myeloproliferative Neoplasm (MPN). After my third bone marrow biopsy, the doctors at Stanford had a slightly different approach.
Their diagnosis is Systemic Mastocytosis with an associated hematological non mast cell disease that is likely chronic eosinophilic leukemia (SM-AHNMD-CEL).
In addition to the vast number of eosinophils in my bone marrow, all of the biopsies have shown aggregates of spindle shaped mast cells. My NIH doc says that the differing diagnoses are semantics. Apparently eosinophils and mast cells hang in the same biological circles, so, in the absence of specific genetic clonality, it is impossible to say whether my disease is primarily eosinophilic with secondary mastocytosis or primarily mastocytosis with secondary eosinophilia.
Welcome to the wonderful world of having an uber rare disease. More than six months in and still no diagnosis. When I ask my docs for any sort of prognosis, they all look at each other and shrug their shoulders with the exception of saying that it will eventually kill me if we don't get it under control. That could be one month or five years.
Either way, we are looking at this new option as a blessing, despite the uncertainty. We are now establishing a path to getting this new drug (midostaurin, which is a TKI that has had limited success in AML patients).
The doctors did not want to leave me off of any treatment as my absolute eosinophil count (AEC) continues to slowly rise. Last CBC I had a WBC of 27 with AEC at 20.5. They started me on hydroxyurea (1 gram/day) to see if that would lower the counts as a bridging strategy to get to midostaurin.
Midostaurin just finished phase II trials and is not approved by the FDA. Novartis is the drug company and they must say yes for compassionate use as well as the FDA, which may take 2 months or more. Then another two months to see if it works. My doctor, who was primary investigator on the trial, is unsure if it will work but thinks it is worth the risk to wait to try. Of the 116 patients in the trial, 5 presented in a similar manner, (SM-AHNMD with CEL). 4 of those 5 had a complete resolution of their eosinophilia.
Additionally, for those of you following the Beat AML initiative, my doctor also sent off my blood for evaluation with Jeff Tyner using the same techniques for AML genome mapping with the hope that another TKI might be better suited for me than even midostaurin.
http://www.lls.org/who-we-are/a-new-approach-to-drug-discovery?src1=35591&src2=
As I have become more and more versed on this subject, I am more and more encouraged by the progress in beating myeologenous leukemias of all forms. I am fairly certain that in 20 years, leukemias of this variety will be treated first with induction, then TKI to vastly reduce chances of relapse and then BMT only in rare occasions. CML has mostly been beaten, replaced by taking a pill with few side effects. We can do this too with AML!
I would encourage all of you out there to write your docs to send any blood that may still be stored to Jeff Tyner and the lab if pathways exist. I remember Dave telling me at the beginning of this journey for me that we are all part of the process to beat this disease. I took that to heart and wholeheartedly agree.
What I would ask of the team for me personally is to continue praying, specifically for the wisdom to make the right choices between BMT and drug therapy, for protection of my heart and other organs from all the eosinophils, for hydroxyurea to work as a bridging strategy, and for midostaurin to be approved for me and to work.
If you have any experience with hydroxyurea or midostaurin, I would appreciate any info as well.
We are so thankful for you all and pray for everyone here daily. Hope and health for everyone.
Vr.
John
Glad to hear from you, I have been wondering how you were doing with the move and getting started on your SCT. Looks like things have taken a turn. Seems like you are in a great place for them to get it figured out.
I am in a clinical trial for Midostaurin, but I am in the standard of care group and not taking the drug. I really don't know anything other than asking about how the patients that were taking the drug were doing. My PA told me that several have had a hard time tolerating the drug and had to be taken off it. But others have done fine on it too. It is strictly used in AML as a inhibitor as you've said to hopefully help in preventing relapse.
Praying for that wisdom for you and your family and the proper treatment to get this cured and done with.
DaveJ
I too am praying that they get a treatment plan figured out for you and get you on the road to recovery and a cure.
Hugs and prayers.
Karen
I am so impressed with your knowledge and research. There are quite a number of TKI out there. My brother Jack had to be put of Hydrea for a while to control the WBC awaiting his clinical trial. Prior to that point he was on Sorafenib (another TKI). There was quite a discussion as his oncologist thought it would help, but the head of the department didn't think it would have an effect. That God that his oncologist went with his gut feeling as the TKI was able to have his counts under control for a few more months as there was very little options left late last year.
As an update, Jack is on a TKI now (asp 2215 a new clinical trial drug AKA Gilteritinib)and is currently going back to UCLA once a week. He is off all steroids which is very good. He has mild GVHD and needs to take a 2.5mg pill once in a while. He's supposedly in remission as there were three negative biopsies but having a very difficult time getting his platelets back to normal. He is needing platelets once every week or every two weeks. ( in fact last week he needed blood as well as platelets)
In general TKI are great with minimal side effects.Thank you for sharing. Will be thinking and praying for you as well as your doctors to guide them in the right direction.