Hi all. This forum has been so helpful to me in the past as my family and I have governed the uncharted territory of myeloma.
My mother was diagnosed last May (2013) with plasma cell leukemia. She had three rounds of DCEP followed by an autologous stem cell transplant in October. Her engraftment took far longer than expected (I think she didn't begin to show signs of engraftment until day 24, but I can't remember exactly).
Anyhow, since then, she has enjoyed six months of good health and a return to near-normal functioning. However, she was now found to have both an orbital plasmacytoma (in and behind her eye, apparently exceedingly rare) and a plasmacytoma in her groin.
It is so confounding because her bloodwork continues to be normal. In the face of normal bloodwork and presumably normal bone marrow, does the presence of these two plasmacytomas qualify as EM relapse?
I would assume yes but want to confirm. From what I've gleaned online, the prognosis is grim once people experience EM relapse - can anyone tell me about their experience with this?
Right now the plan is 5 weeks of radiation followed by pomalidomide [Pomalyst].
Thank you all so much.
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6 posts
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Re: What exactly is extramedullary relapse?
While I know next to nothing regarding leukemia, I do know a bit about extramedullary (outside the bone) plasmacytomas, as that is how my multiple myeloma was diagnosed. A big firm "mass" in my front left forehead -- like a raised bump -- that ate through part of my skull.
My plasmacytoma "presented" in my left frontal sinus (above the left eyebrow--and NOT in the bone) and grew / extended down into my left orbit, and inferiorly, into the area next to the dura of my brain (no invasion) (some would say that is completely impossible, as I have NO brain)
Oh well.
I underwent surgery in May 2012 -- utilizing Iowa Head & Neck Protocols. They cut you ear to ear -- over the top, they peel your scalp and face down to your mouth, cut the leathery galea, peel it back, cut out the front skull bone, remove the tumor and the adjacent areas --"MARGINS", and then re-construct you with titanium and hydroxyapatite (bone cement), etc --then flap your galea and scalp / face back in place and staple you together). It's on Youtube.
Kinda like fixing a car fender, once the tumor is removed, actually.
After healing, I underwent 9 weeks of radiation (45 GY) to skull and hip. (Lesion suspected there too).
No re-growth on MRI /CT scans.
Standard induction -Velcade / dex used from late October to February.
Stem prep / collection / apheresis thru late March 2013. ( No stem cell transplant undertaken)
Maintenance with low dose Revlimid since.
Growth near tragal cartilage in September 2013 (ear) was an infection -- cleared with antibiotics.
Total hip replacement in February 2014. (Yep, radiation is damn hard on hips.)
No recurrence to date of tumors.
I am a t(11,14) by FISH. A GOOD karyotype of multiple myeloma to have. Last M Spike measurement was 0.1 g/dL. (Pretty darn good.) 0.0 g/dl would be perfect (and "normal").
True recurrent EMP's in multiple myeloma can be a poor prognostic factor (see literature).
Many times suspected EMPs are something else, or are a collection of cells or inflammatory process, secondary to infections or lesions elsewhere. Biopsy would be a prudent thing to do.
I was freaking out regarding growth in Sept 2013. It turned out to be just an inflamed lymph node / localized infection. Didn't need the biopsy, after all. Not an EMP.
Since your mom has leukemia, her mileage may definitely vary and EMP may not be the correct biophysical model to analyze with??
Call her MD and have a straight talk, methinks.
Good luck.
My plasmacytoma "presented" in my left frontal sinus (above the left eyebrow--and NOT in the bone) and grew / extended down into my left orbit, and inferiorly, into the area next to the dura of my brain (no invasion) (some would say that is completely impossible, as I have NO brain)
Oh well.
I underwent surgery in May 2012 -- utilizing Iowa Head & Neck Protocols. They cut you ear to ear -- over the top, they peel your scalp and face down to your mouth, cut the leathery galea, peel it back, cut out the front skull bone, remove the tumor and the adjacent areas --"MARGINS", and then re-construct you with titanium and hydroxyapatite (bone cement), etc --then flap your galea and scalp / face back in place and staple you together). It's on Youtube.
Kinda like fixing a car fender, once the tumor is removed, actually.
After healing, I underwent 9 weeks of radiation (45 GY) to skull and hip. (Lesion suspected there too).
No re-growth on MRI /CT scans.
Standard induction -Velcade / dex used from late October to February.
Stem prep / collection / apheresis thru late March 2013. ( No stem cell transplant undertaken)
Maintenance with low dose Revlimid since.
Growth near tragal cartilage in September 2013 (ear) was an infection -- cleared with antibiotics.
Total hip replacement in February 2014. (Yep, radiation is damn hard on hips.)
No recurrence to date of tumors.
I am a t(11,14) by FISH. A GOOD karyotype of multiple myeloma to have. Last M Spike measurement was 0.1 g/dL. (Pretty darn good.) 0.0 g/dl would be perfect (and "normal").
True recurrent EMP's in multiple myeloma can be a poor prognostic factor (see literature).
Many times suspected EMPs are something else, or are a collection of cells or inflammatory process, secondary to infections or lesions elsewhere. Biopsy would be a prudent thing to do.
I was freaking out regarding growth in Sept 2013. It turned out to be just an inflamed lymph node / localized infection. Didn't need the biopsy, after all. Not an EMP.
Since your mom has leukemia, her mileage may definitely vary and EMP may not be the correct biophysical model to analyze with??
Call her MD and have a straight talk, methinks.
Good luck.
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Rneb
Re: What exactly is extramedullary relapse?
Wow thanks for the in depth info, Rneb. No two cases are alike when it comes to myeloma, that's for sure.
My mom's masses were already confirmed to be plasmacytomas (the groin via a biopsy and the eye was deemed to be one de facto based on the groin biopsy). She will have simultaneous eye and hip radiation. Just really hoping that there might still be some hope for good health (relatively speaking) in her future, as I would hate to see her go through all this pain and suffering for naught. At the same time, however, I want to be practical and prepared.
My mom's masses were already confirmed to be plasmacytomas (the groin via a biopsy and the eye was deemed to be one de facto based on the groin biopsy). She will have simultaneous eye and hip radiation. Just really hoping that there might still be some hope for good health (relatively speaking) in her future, as I would hate to see her go through all this pain and suffering for naught. At the same time, however, I want to be practical and prepared.
Re: What exactly is extramedullary relapse?
Indeed periorbital and groin disease are considered extramedullary disease (EMD). EMD means that the myeloma cells are capaple of growing independent of the bone marrow, which can be thought of as a nourishing environment for myeloma cells. So these cells are a bit wiser and stronger and at time can be challenging to treat.
However, as was the case describe in the initial response to your post, many times with radiation and systemic therapy EMD can be kept at bay. And it sounds like your mom has gotten relatively little systemic therapy. So she really may benefit from the pomalidomide [Pomalyst] and or proteasome inhibitor therapy (bortezomib [Velcade] or carfilzomib [Kyprolis]). But it's always prudent to be cautiously optimistic.
However, as was the case describe in the initial response to your post, many times with radiation and systemic therapy EMD can be kept at bay. And it sounds like your mom has gotten relatively little systemic therapy. So she really may benefit from the pomalidomide [Pomalyst] and or proteasome inhibitor therapy (bortezomib [Velcade] or carfilzomib [Kyprolis]). But it's always prudent to be cautiously optimistic.
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Dr. Heather Landau - Name: Heather Landau, M.D.
Beacon Medical Advisor
Re: What exactly is extramedullary relapse?
Thanks so much Dr. Landau. I can't tell you how much I appreciate the feedback.
I guess what I'm confused about is the mechanism of cell proliferation in generating plasmacytomas. I would think that there would have to be marrow and/or blood involvement before a plasmacytoma could be formed. I have been trying to read all of the literature available online and from what I could find it seems like, in the absence of marrow progression, these extramedullary areas could have been sanctuary sites that were somehow untouched by intensive chemo. Or that in patients with extramedullary relapse there is a shift from secretion of intact immunoglobulins to free light chains only (is this the same as nonsecretory disease?).
It's just hard to get a handle on what was the cause/mechanism of this relapse. We were feeling so encouraged by unremarkable bloodwork and then all of a sudden these masses appeared.
I guess what I'm confused about is the mechanism of cell proliferation in generating plasmacytomas. I would think that there would have to be marrow and/or blood involvement before a plasmacytoma could be formed. I have been trying to read all of the literature available online and from what I could find it seems like, in the absence of marrow progression, these extramedullary areas could have been sanctuary sites that were somehow untouched by intensive chemo. Or that in patients with extramedullary relapse there is a shift from secretion of intact immunoglobulins to free light chains only (is this the same as nonsecretory disease?).
It's just hard to get a handle on what was the cause/mechanism of this relapse. We were feeling so encouraged by unremarkable bloodwork and then all of a sudden these masses appeared.
Re: What exactly is extramedullary relapse?
Perhaps if you think in terms of "Inhaled pesticides"...you might understand the pathway to the Plasmacytoma near your mom's eye.
Upper Aero-digestive Plasmacytomas comprise 80% of EMP's.
As Plasmacytoma's are , to my understanding, nothing more than a shell membrane containing a collection of aberrant cells replicating redundant Proteins, a rich blood supply may be all that is required for them to take root ? That would explain a high percentage of tumors in the Head & Neck regions. (Highly vascular)
BTW--My 3 Bone Biopsies and my 24 Urine catches (Bence-Jackson Proteins) have all been negative.( ie. Should I even have multiple myeloma ?)
However, My SPEP's have been accurate. (Low) My Light chains have been accurate.( Also low)
Weird disease.
Why did we develop EMP's in the Sinus regions ?? I suspect that the Immune system may just be stronger in the Bone marrow, than the Sinus cavities..?
Good luck to your Mom.
Upper Aero-digestive Plasmacytomas comprise 80% of EMP's.
As Plasmacytoma's are , to my understanding, nothing more than a shell membrane containing a collection of aberrant cells replicating redundant Proteins, a rich blood supply may be all that is required for them to take root ? That would explain a high percentage of tumors in the Head & Neck regions. (Highly vascular)
BTW--My 3 Bone Biopsies and my 24 Urine catches (Bence-Jackson Proteins) have all been negative.( ie. Should I even have multiple myeloma ?)
However, My SPEP's have been accurate. (Low) My Light chains have been accurate.( Also low)
Weird disease.
Why did we develop EMP's in the Sinus regions ?? I suspect that the Immune system may just be stronger in the Bone marrow, than the Sinus cavities..?
Good luck to your Mom.
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Rneb
6 posts
• Page 1 of 1