My brother was diagnosed with a rare, aggressive non-Hodgkin's lymphoma (Subcutaneous Panniculitis-Like T-Cell Lymphoma or SPTCL to be exact) coupled with HLH, is this 100% curable? What is the survival rate of this combination?

aqb:
I'm sorry to read of your brother's dx. I couldn't find any examples of this dual diagnosis but I did find some info on SPTCL, specifically in a 2009 issue of the journal Archives of Pathology and Laboratory Medicine, regarding treatment and prognosis:

"Most patients with aggressive disease who fail with initial immunosuppressive therapy [ie high-dose systemic corticosteroids] are treated with anthracycline- based and anthracenedione-based chemotherapy with combined cyclophosphamide, doxorubicin, vincristine, and prednisone [ie the CHOP regimen]. In localized disease without hemophagocytic syndrome, radiation can produce longterm remissions. In addition, radiation therapy can be used for palliative purposes. High-dose chemotherapy and stem cell transplantation has been shown to be very effective, with a high complete remission rate. Therefore, high-dose chemotherapy and stem cell transplantation is usually considered in primary refractory or recurrent cases."

That much would suggest that it's treatable, but the disease has only been recognized since 1991, and considering how rare it is, there's not bound to be much information out there.

Clearly since your brother is presenting with hemophagocytic syndrome that would eliminate radiation therapy. I did find an article on HLH that has a lot of stats; check out:

Fisman, David N.. "Hemophagocytic Syndromes and Infection.(Statistical Data Included)." Emerging Infectious Diseases. U.S. National Center for Infectious Diseases. 2000.

To anyone who has a loved one with similar symptoms (not as a guide, but more of a message to never give up), my brother went through the CHOP regimen - that was unsuccessful. He grew weaker, and eventually went in a coma for a few days. Doctors recommended for us to pull the plug on him, but miraculously he woke up the day it was supposed to happen. From then on he had to recover not only the failed chemo treatment, but infection that got to his legs.

This went on for a couple months, once he was healthy enough they treated him with GEMZAR. It seems to have worked, he is healthier, has no life support, breathing tube, or feeding tube anymore. Mostly in bed, he is able to walk with a walker now, and hopefully the next few sessions will put this cancer in full remission.

Through all of this, it seems like the best medicine was the love and support from his family and friends.

Best wishes and God bless to all families out there going through this.

Thank you for the update, and that's wonderful news about your brother. I wish him continued good health, and I extend that to your family as well.

Hello aqb,
I was just diagnosed with SPTCL 4 months ago, a few weeks after i gave birth. I sought treatment for it at a cancer center in california, and was given the run-around for a month to get an appointment with a oncologist. After i did see her, she told me that most live with this disease with no problem and fatality was uncommon. I have several symtoms, including severe anemia (hemoglobin 3) the doctor told me the cancer was completly managable, and all my symptoms were from the anemia she sent me home with instructions to take viatmins. I am currently desperatly searching for an oncologist who has some experience with SPTCL, because the first doctor has no idea what the hell she was talking about. I would be very interested to hear how your brother is doing, and i hope it will be good news. Thank you

apyleoflove:
so sorry to read of your dx. In cases like this, where the lymphoma subtype is rare, the best way to find someone who knows something about it is to search the subtype name at PubMed and see who is publishing research on this disease. You can also try an NCCN hospital that has a cutaneous lymphoma department, like Stanford's Multidisciplinary Cutaneous Lymphoma Group. At the very least, if they can't directly help you they will know who can.

Ross

I really don't have much idea about this combination, but, my cousin was treated with the chemotherapy process, which led to a successful surgery and he is in way to recover the disease. People usually fear chemotherapy, directing it to be one of the most dangerous therapy as well. A lot of patient loose their lives while processing this treatment. A medical alert system was recommended to my cousin after the treatment for about couple of weeks. The therapy cause severe joints pain after the treatment. So, it is better to keep alert systems on the recovery period.

Hi All,
I was diagnosed with Stage 2 SPTCL back on Dec 2009. I know it's been frustrating trying to find any kind of positive information about this Cancer. I was fortunate that my doctor referred my case to Stanford. I've had already 4 different treatments. BUT all of them I haven't lost my hair or had not even a quarter of the side effects.
Today I hopefully finished the last of my Chemo treatments. I was first told that I would need at least 6 months of treatment. I just completed 4 months. The Chemo they have me on is a new drug that was FDA approved in Nov of 2009. I'm the first SPTCL patient to ever have this treatment. It seems to be working. Almost all of the nodules are about 80% smaller.
Please know that there is hope on finding a cure for us.

Keep positive!

Monica

Hey Monica,
Where you dx with SPTCL aplha/beta or gamma/delta? I have alpha/beta and I'm interested to see if chemo is used for the this type, or just the more aggressive gamma/delta.
-thanks danielle

@apyleoflove: my brother's GEMZAR treatment got him well enough to have him transferred back home from Nor Cal to So Cal. He had a few healthy weeks where he spent quality time with family and friends, but was eventually set up to go to the City of Hope hospital where they specialize in cancer research.

He recently went through a combination of radiation therapy as well as chemotherapy. He described it as not as easy as it sounds; however, it was much more comforting because this time he could walk, and for the most part take care of himself. What made this stressful was that they put him back on the CHOP regimen, the chemotherapy that initially put him in a coma for a couple of days. The doctors believe that this is the treatment that will have the best success rate. In addition to all this, all of my siblings had our blood tested for a possible bone marrow match - unfortunately, none of us were a match and he had to go through a process where they extracted his bone marrow and put it back in him (I forgot what the exact term for this was).

My brother is back home recovering from that, and seems to be getting healthier. Going through a series of tests to see how successful that treatment was.

@Monica: What is the name of the treatment your doctors are putting you on if I may ask?

To the both of you, best wishes and stay strong!

Aqb,
I'm really happy to hear that he is staying strong, this disease is a lot harder to deal with then most realize. I'm not currently undergoing any treatment as I'm trying to find a specialist that accepts my insurance. I just recently got authorization at UCLA with a doctor I was referred to by the cutaneous lymphoma foundation. I tried to get into city of hope because its closer to me, but they didn't accept my insurance. I don't feel well enough to make the drive from Arizona to California right now, I'm blacking out and extremely weak so I'm afraid to drive on my own with my baby, and my husband works 6 days a week. So I planning to see a hematologist in my home town, get some iron/b12 shots see if I can boost myself up before traveling to UCLA. Aqb, dose your brother have alpha/beta or gamma/delta?
-danielle