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Thread: Peripheral T-Cell Lymphoma, Not Otherwise Specified

  1. #1
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    Peripheral T-Cell Lymphoma, Not Otherwise Specified

    Dear members/readers of this forum,

    I am a 33 year old man and I’d like to share some details of my own battle with a rare case of PTCL NOS, hoping that someone may somehow benefit from this. I'd like to say that I am very grateful to my haematologist and all the other doctors who worked on my case and managed to identify and treat this disease effectively.

    Clinical details:
    Rapidly growing tumor on upper right thigh. Started as a painless red spot/lump that reached 5cm in max diameter and 2-3cm in max height within 7-8 weeks of presentation. Small red spots/lumps later appeared on both buttocks.

    Histological details & diagnosis:
    Medium-sized malignant T-cells. Perivascular and periappendageal distribution. Notable vascularity and tissue breakdown.
    Immunophenotype: CD2+, CD3+, CD4+, CD5+, CD7-, CD8-, TIA-1+, GrB+/-, CD30-, BCL6-.
    Very high proliferation rate: Ki67+ > 75%.
    Monoclonal rearrangement of TCR beta and gamma genes.
    Few B-cells and very few CD8+ T-cells in the tumor’s microenvironment.
    CT scan & bone marrow biopsy both negative for lymphoma.
    Three pathologists (including a world expert on cutaneous lymphomas) agreed on the diagnosis “primary cutaneous PTCL NOS”.

    Treatment & outcome:
    Induction therapy with 6 cycles of CHOP, followed by consolidation therapy with BEAM + autologous stem cell rescue. No irradiation at any point.
    Complete remission after the end of the first CHOP cycle which continues to this day, almost 14 months later.

    Remark:
    The GP originally suspected a skin infection and gave me the antibiotic clarithromycin 2 x 500mg daily for a week. Slowly but visibly the tumour shrank during that week. I was later referred to the local hospital for a biopsy. When the tumor started to grow back after the biopsy, and while I was still waiting for the results, I asked for a second, longer course of clarithromycin. This led to the complete eradication of the primary tumor on the thigh, although it did not prevent the metastasis to the buttocks. I later discovered that this drug has notable antineoplastic activity in general and antilymphoma activity in particular, through various mechanisms, and that it is currently being tested in combination with other drugs.
    Last edited by t-cell; 09-14-2015 at 03:06 PM.

  2. #2
    Super Moderator Top User po18guy's Avatar
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    Quite a story! Congratulations on your remission. A good thing that the biopsy sample went to a competent pathologist. I am surprised that it responded so well to CHOP, as that regimen (with a few exceptions) has a poor record against T-Cell Lymphomas. Did you have the BEAM, or was treatment stopped once remission was achieved?

    Many antibiotic drugs show a certain level of effectiveness against cancers, as they also target rapidly dividing (bacterial) cells. Occasionally, there is sufficient crossover to show some effectiveness against a particular cancer. With the noted increased vascularity, and the involvement of B-Cells in the tumor, I would almost imagine that your PTCL-NOS was similar to AngioImmunoblastic T-Cell Lymphoma, as that version demonstrates B-Cell involvement as well as angiogenesis - the formation of new blood veins around the tumor cells.

    I am a little confused as to why the CT scan would be negative, when the tumor was clearly visible and palpable(?)
    05/08-07/08 Tumor appears behind left ear. Followed by serial medical incompetence on the parts of PCP, veteran oncologist and pathologist (misdiagnosis via non-diagnosis). Providential guidance to proper care at an NCI designated comprehensive cancer center.
    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 TEC (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 and precursor to Acute Myeloid Leukemia.
    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 measurable - 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. Narrow-band UV-B therapy started, but discontinued for lack of response. One treatment of P-UVAreceived, but halted due to medication reaction.
    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. 8 year old Power Port removed and replaced with Vortex (Smart) Port for ECP.
    05/2017 Chronic anemia (low hematocrit). Chronic kidney disease. Cataracts from radiation and steroids.
    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.
    03/18 Began 400mg daily of KD025, a rho-Associated Coiled-coil Kinase 2 Inhibitor (ROCK2).
    09/18 Due to refractory GvHD, Extracorporeal Photopheresis halted after 15 months ue to lack of additional benefit.
    10/18 I was withdrawn from the Kadmon KD025 clinical trial due to increasing fatigue/lack of benefit.
    11/18 Began therapy with Ruxolitinib (Jakafi), a JAK 1&2 inhibitor class drug. Started at half-dose due to concerns with drug interactions.

    To date: 1 cancer, relapse, second relapse/mutation into 2 cancers, then 3 cancers simultaneously, 20 chemotherapy/GVHD drugs in 11 regimens (4 of them at least twice), 5 salvage regimens, 4 clinical trials, 5 post-transplant immuno-suppressant/modulatory drugs, the equivalent of 1,000 years of background radiation from 40+ CT series scans and about 24 PET scans.
    Both lymphoid and myeloid malignancies lend a certain symmetry to the hematological journey.

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

  3. #3
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    Thanks for your reply.

    The CT scan was negative for systemic or visceral lymphoma, i.e. the disease was only on the skin.
    Given that the cells were BCL6- I don't think they were very intimately related to AITL cells, but who knows. I mentioned the small numbers of B-cells and CD-8 T-cells that were seen in the tumor's background because these cells are often useful in distinguishing between PTCL NOS (low numbers) and CD4+ pleomorphic small/medium sized T-cell lymphoma (large numbers). The latter is a far more indolent condition and does not require aggressive treatment.

    I was lucky that CHOP worked for me. It seems to be working (partly or fully) for roughly 50% of the patients with PTCL NOS, while 50% of those for whom it does result in complete remission eventually relapse. Hence the decision to consolidate the good response with BEAM and auto-transplant in my case. My stem cells were mobilized with cyclophosphamide + pegfilgastrim two months after the last CHOP, and the transplant was carried out two months later. I was kept in for three weeks. Almost six months after the transplant everything has been normalized except for the red blood cells which are borderline.

    I've read your profile. Quite a journey! You most definitely have my respect and my very best wishes!

  4. #4
    Super Moderator Top User po18guy's Avatar
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    You have obviously taken quite an interest in your disease, as is evidenced by your knowledge of the various proteins involved. What I missed in your post was that it was confined to the skin, but I did catch that it had a very high proliferation rate. Of course, the danger in that is that it would very likely become systemic and the battle would then have to be fought on a larger scale. I wondered about any similarity to AITL since increased vascularity was noted in your report. That is one of the hallmarks of AITL. As well, in my case, AITL's mixture of mutated T-Cells and abnormal B-Cells apparently confounded the first pathologist and contributed to my initial misdiagnosis of no malignancy found. After that, I sought out a world-class facility and am still here over seven years later.

    Yes, there are other CHOP success stories here but they tend to be rare. One was in combination with radiation. The autologous transplant at first remission is a good chance for a long-term remission, or potential cure, as I am sure you know. I was not able to achieve a first remission long enough to collect my stem cells for transplant. Thus, we had to take the research/clinical trial route. However, we were reaching the end of the list of viable lymphoma drugs.

    In the end, we exercised the "nuclear option" and I received a "moderate" intensity haploidentical allogeneic transplant.
    05/08-07/08 Tumor appears behind left ear. Followed by serial medical incompetence on the parts of PCP, veteran oncologist and pathologist (misdiagnosis via non-diagnosis). Providential guidance to proper care at an NCI designated comprehensive cancer center.
    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 TEC (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 and precursor to Acute Myeloid Leukemia.
    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 measurable - 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. Narrow-band UV-B therapy started, but discontinued for lack of response. One treatment of P-UVAreceived, but halted due to medication reaction.
    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. 8 year old Power Port removed and replaced with Vortex (Smart) Port for ECP.
    05/2017 Chronic anemia (low hematocrit). Chronic kidney disease. Cataracts from radiation and steroids.
    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.
    03/18 Began 400mg daily of KD025, a rho-Associated Coiled-coil Kinase 2 Inhibitor (ROCK2).
    09/18 Due to refractory GvHD, Extracorporeal Photopheresis halted after 15 months ue to lack of additional benefit.
    10/18 I was withdrawn from the Kadmon KD025 clinical trial due to increasing fatigue/lack of benefit.
    11/18 Began therapy with Ruxolitinib (Jakafi), a JAK 1&2 inhibitor class drug. Started at half-dose due to concerns with drug interactions.

    To date: 1 cancer, relapse, second relapse/mutation into 2 cancers, then 3 cancers simultaneously, 20 chemotherapy/GVHD drugs in 11 regimens (4 of them at least twice), 5 salvage regimens, 4 clinical trials, 5 post-transplant immuno-suppressant/modulatory drugs, the equivalent of 1,000 years of background radiation from 40+ CT series scans and about 24 PET scans.
    Both lymphoid and myeloid malignancies lend a certain symmetry to the hematological journey.

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

  5. #5
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    Still in complete remission, >4 years post-transplant.

  6. #6
    Super Moderator Top User po18guy's Avatar
    Join Date
    Feb 2012
    Posts
    10,307
    Excellent! Thank you for checking back in. Right behind you at 4 years next month.

 

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