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Thread: Atypical Chronic Myloid Leukemia

  1. #1
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    Atypical Chronic Myloid Leukemia

    Does anyone here have, or know anyone who has, Atypical Chronic Myeloid Leukemia? It is very rare. It is not really a type of CML, but more an "overlap" hybrid disease (MDS/MPN) that has some features like Myelodysplastic disease (declines in some blood counts - like platelets...leading to thrombocytopenia) and some Myeloproliferative neoplasms (increases in white blood cells/neutrophils). It "looks" like CML, but has no "Philadelphia" chromosome (i.e. Is Ph-, or Philadelphia negative). It is generally called a Myeloproliferative disease (MPN), though it is really an MDS/MPN. The World Health Organization (WHO) classifies it as a subtype of MDS. It does not respond to Gleevec (imatinib), as CML does, nor well to any other drugs, and has a grim prognosis (12-24 mos). Bone marrow transplant/hemopoietic stem cell transplant (BMT/HSCT) is the only potentially curative option. I have it. ACML

  2. #2
    Super Moderator Top User po18guy's Avatar
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    Welcome, but sorry to hear of your situation. It seems that we have only leukemia/MDS folks here with anomalous cases. Prior to transplant (haplo-allo), I had 26% 20q- MDS in my marrow. It is low-risk, but the percentage was worrisome. I would imagine that your options are a clinical trial, if one exists, or a transplant. Having had a transplant, I tend to view it as a last gasp, and a trial would be much preferable. I was able to locate completed ACML studies, and a few that are or will be recruiting. The pickings are rather sparse, but it only takes one.

    https://www.clinicaltrials.gov/ct2/r...e=&city=&dist=

    I have just been "consented" for my fourth clinical trial, with two being for disease and two being for chronic-Graft-versus-Host-Disease (cGvHD). If you have not, please take a look at the trials. I am here only because such a trial was available early in 2009. At that point, my prognosis was "extremely poor" and has dropped several times since. In October, an infectious disease doctor opined that I had about a 0.5% chance of being alive going into my 10th year.

    Don't lose hope!
    07/08 Age 56 DX 1) Peripheral T-Cell Lymphoma-Not Otherwise Specified. Stage IV-B, >50 ("innumerable") tumors, bone marrow involvement.
    08/08-12/08 Four cycles CHOEP14 + four cycles GND (Cyclofosfamide, Doxorubicin, Vincristine, Etoposide, Prednisone & Gemcitabine, Navelbine, Doxil)
    02/09 2) Relapse.
    03/09-06/13 Clinical trial of Romidepsin > long-term study. NED for 64 twenty-eight day cycles, dose tapered.
    07/13 3) Relapse, 4) Suspected Mutation.
    08/13-02/14 Romidepsin increased, stopped for lack of response. Watch & Wait.
    09/14 Relapse/Progression. Visible cervical nodes appear within 4 days of being checked clear.
    10/06/14 One cycle Belinostat. Discontinued to enter second clinical trial.
    10/25/14 Clinical trial of Alisertib/Failed - Progression.
    01/12/15 Belinostat resumed/Failed - Progression. 02/23/15
    02/24/15 Pralatrexate/Failed - Progression. 04/17/15
    04/15 Genomic profiling reveals mutation into PTCL-NOS + AngioImmunoblastic T-Cell Lymphoma. Stage IV-B a second time. Two dozen tumors + small intestine (Ileum) involvement.
    04/22/15 TREC (Bendamustine, Etoposide, Carboplatin). Full response in two cycles. PET/CT both clear. Third cycle followed.
    06/15-07/15 Transplant preparation (X-rays, spinal taps, BMB, blood test, MUGA scan, lung function, CMV screening, C-Diff testing etc. etc. etc.) Intrathecal Methotrexate during spinal tap.
    BMB reveals 5) 26% blast cells of 20q Deletion Myelodysplastic Syndrome MDS), a bone marrow cancer.
    07/11-12/15 Cyclofosfamide + Fludarabine conditioning regimen.
    07/16/15 Total Body Irradiation.
    07/17/15 Moderate intensity Haploidentical Allogeneic Stem Cell Transplant receiving my son's peripheral blood stem cells.
    07/21-22/15 Triple dose Cyclofosfamide + Mesna, followed by immunosuppressants Tacrolimus and Mycophenolate Mofetil.
    07/23-08/03/15 Marrow producing zero blood cells. Fever. Hospitalized two weeks.
    08/04/15 Engraftment occurs, and blood cells are measureable - released from hospital.
    08/13/15 Day 26 - Marrow is 100% donor cells. Platelets climbing steadily, red cells follow.
    09/21/15 Acute skin Graft versus Host Disease arrives.
    DEXA scan reveals Osteoporosis.
    09/26/-11/03/15 Prednisone to control skin GvHD.
    11/2015 Acute GvHD re-classified to Chronic Graft versus Host Disease.
    05/2016 Tacrolimus stopped. Prednisone from 30-90mg daily tried. Sirolimus begun.
    09/16/16 Three skin punch biopsies.
    11/04/16 GvHD clinical trial of Ofatumumab (Arzerra) + Prednisone + Methylprednisolone begun.
    12/16 Type II Diabetes, Hypertension - both treatment-related.
    05/17 Extracorporeal Photopheresis (ECP) begun in attempt to control chronic Graft-versus-Host-Disease (cGvHD.
    06/17 Trying various antibiotics in a search for tolerable prophylaxis.
    08/17 Bone marrow biopsy reveals the presence of 2% cells with 20q Deletion Myelodysplastic Syndrome, considered to be Minimum Residual Disease.
    12/17 Bone marrow biopsy reveals no abnormalities in the marrow - MDS eradicated. The steroid taper continues.
    01/18 Consented for Kadmon clinical trial of drug KD025, a ROCK2 inhibitor that is believed to help with chronic GvHD.
    To date: 18 chemotherapeutic drugs in 9 regimens (4 of them at least twice), 5 salvage regimens, 3 clinical trials, 4 post-transplant immuno-suppressant drugs, the equivalent of 1,000 years of background radiation from scanning from 45+ CT series scans and about 24 PET scans. Two lymphoid malignancies plus a myeloid malignancy lend a certain symmetry to the journey.

    Believing in the redemptive value of suffering makes all the difference.

  3. #3
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    po18guy,

    Thank you for your reply, and I should tell you that you are the particular person I first read about here and that drew me into registering. I found my way to you or focused in on you because I was particularly looking for haplo transplant patients who had used a son (presumably an adult son), because I am potentially in that situation, though I am very (I would say adamantly) averse to pulling my son into this and hurting him in any way. I realize rationally that, from a medical standpoint, there is probably little risk of lasting damage and transplant doctors view this as temporary discomfort for the donor. But personally, and for a variety of reasons, I am just very averse to using him as a donor - especially the 100+ hip bone punctures from a BMT, but also even the effects and possible risks of a peripheral blood stem cell harvest.
    I have no really good other donors unfortunately (though you seemed to have even less) - 3 sibs (younger sister (67), then brother (62), then sister (55), but brother is only even partially close match - a 5/6. But he is 62 and doctors really want a young guy - better stem cells. Next sister had breast cancer 4 years ago - lumpectomy, radiation, chemo - and so was ruled out; but tragically also got mantle cell lymphoma about a week after her 3rd cancer free anniversary from breast cancer, and about a month after discovery of my ACML, and just died less than a year later just before Thanksgiving after failure of 6 cycles of R-BAK, failure trial of imbrutinib, one last gasp of R-CHOP, and 6 weeks short of CAR-T trial. My youngest sister is a haplo 4/6, is female, and has had kids - all strikes against her. In the Be The Match database, I only have 9/10 or 7/8 donors - mismatches. So my mid-20's son is suggested as perhaps best possibility - as a haplo. But I absolutely do not want to use him or even involve him - I don't want to cause him pain, or damage him in any way, now or down the road, especially just to get a few more *possible* (and not even certain at all - transplant could go badly, relapse, cause miserable or even fatal GVHD.....docs would probably do reduced intensity at my age of 70, and that has its own complexities and problems - trying to get GVL, trying not to have relapse, trying to manage infection from suppressed immune system, etc. - and as you said, things like allergies from your donor, even own son ) years of my life. I would almost rather just die of the cancer - accept that it is my time - then use him. But I am trying to be rational and consider privately. So, aside from your own very long and hard and brave road, I wanted to ask how it went for your son? Whether he had, or you see now, or worry about any problems for him? Docs all say its no risk for the donor (and they are trying to recruit volunteers), and mostly it probably is not - but there are articles out there documenting that there have been *some* adverse events for donors.

  4. #4
    Super Moderator Top User po18guy's Avatar
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    Thank you for your kind words. First, I would caution you not to over-think this. Each and every person who volunteers to donate marrow or stem cells does so out of a certain amount of love. Both the marrow as well as the stem cells are easily replaced by the body, as it seeks its natural equilibrium. The only pain involved is perhaps some bone pain from the Colony Growth Stimulation Factor that is injected, and from the venous access during the collection process. Both are transient and short-lived. My son advised me that he would have donated even if it caused permanent damage.

    Back in 2011, my daughter held a marrow donation registry. One man who signed up was simply passing by and registered as he throught it was simply the right thing to do. A cohesive, coherent society is made up of such.

    Love is the controlling factor. People donate out of love of family, of friend, of neighbor - even the unknown neighbor.
    07/08 Age 56 DX 1) Peripheral T-Cell Lymphoma-Not Otherwise Specified. Stage IV-B, >50 ("innumerable") tumors, bone marrow involvement.
    08/08-12/08 Four cycles CHOEP14 + four cycles GND (Cyclofosfamide, Doxorubicin, Vincristine, Etoposide, Prednisone & Gemcitabine, Navelbine, Doxil)
    02/09 2) Relapse.
    03/09-06/13 Clinical trial of Romidepsin > long-term study. NED for 64 twenty-eight day cycles, dose tapered.
    07/13 3) Relapse, 4) Suspected Mutation.
    08/13-02/14 Romidepsin increased, stopped for lack of response. Watch & Wait.
    09/14 Relapse/Progression. Visible cervical nodes appear within 4 days of being checked clear.
    10/06/14 One cycle Belinostat. Discontinued to enter second clinical trial.
    10/25/14 Clinical trial of Alisertib/Failed - Progression.
    01/12/15 Belinostat resumed/Failed - Progression. 02/23/15
    02/24/15 Pralatrexate/Failed - Progression. 04/17/15
    04/15 Genomic profiling reveals mutation into PTCL-NOS + AngioImmunoblastic T-Cell Lymphoma. Stage IV-B a second time. Two dozen tumors + small intestine (Ileum) involvement.
    04/22/15 TREC (Bendamustine, Etoposide, Carboplatin). Full response in two cycles. PET/CT both clear. Third cycle followed.
    06/15-07/15 Transplant preparation (X-rays, spinal taps, BMB, blood test, MUGA scan, lung function, CMV screening, C-Diff testing etc. etc. etc.) Intrathecal Methotrexate during spinal tap.
    BMB reveals 5) 26% blast cells of 20q Deletion Myelodysplastic Syndrome MDS), a bone marrow cancer.
    07/11-12/15 Cyclofosfamide + Fludarabine conditioning regimen.
    07/16/15 Total Body Irradiation.
    07/17/15 Moderate intensity Haploidentical Allogeneic Stem Cell Transplant receiving my son's peripheral blood stem cells.
    07/21-22/15 Triple dose Cyclofosfamide + Mesna, followed by immunosuppressants Tacrolimus and Mycophenolate Mofetil.
    07/23-08/03/15 Marrow producing zero blood cells. Fever. Hospitalized two weeks.
    08/04/15 Engraftment occurs, and blood cells are measureable - released from hospital.
    08/13/15 Day 26 - Marrow is 100% donor cells. Platelets climbing steadily, red cells follow.
    09/21/15 Acute skin Graft versus Host Disease arrives.
    DEXA scan reveals Osteoporosis.
    09/26/-11/03/15 Prednisone to control skin GvHD.
    11/2015 Acute GvHD re-classified to Chronic Graft versus Host Disease.
    05/2016 Tacrolimus stopped. Prednisone from 30-90mg daily tried. Sirolimus begun.
    09/16/16 Three skin punch biopsies.
    11/04/16 GvHD clinical trial of Ofatumumab (Arzerra) + Prednisone + Methylprednisolone begun.
    12/16 Type II Diabetes, Hypertension - both treatment-related.
    05/17 Extracorporeal Photopheresis (ECP) begun in attempt to control chronic Graft-versus-Host-Disease (cGvHD.
    06/17 Trying various antibiotics in a search for tolerable prophylaxis.
    08/17 Bone marrow biopsy reveals the presence of 2% cells with 20q Deletion Myelodysplastic Syndrome, considered to be Minimum Residual Disease.
    12/17 Bone marrow biopsy reveals no abnormalities in the marrow - MDS eradicated. The steroid taper continues.
    01/18 Consented for Kadmon clinical trial of drug KD025, a ROCK2 inhibitor that is believed to help with chronic GvHD.
    To date: 18 chemotherapeutic drugs in 9 regimens (4 of them at least twice), 5 salvage regimens, 3 clinical trials, 4 post-transplant immuno-suppressant drugs, the equivalent of 1,000 years of background radiation from scanning from 45+ CT series scans and about 24 PET scans. Two lymphoid malignancies plus a myeloid malignancy lend a certain symmetry to the journey.

    Believing in the redemptive value of suffering makes all the difference.

 

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