I will make this list larger. You can help by commenting below.
- Introduction and prognosis
- Symptoms of BPDCN
- Where to go for treatment
- Where to go for financial help
- Can you do anything in advance?
Overview of BPDCN
First, don't be frightened by what you read on the internet. A lot of what you will find is terrifying but is, in young people slang, "so 2006."
Because of the success rates of bone marrow transplants, the survival rate of BPDCN patients has gone from basically zero—it's possible that 1 of the 200 or so patients diagnosed with BPDCN has survived without a marrow transplant—to at least 25%. Those are not great odds, but they get better the younger you are.
At this point, it appears to me from studies and experience—almost everyone diagnosed with BPDCN gets hold of me nowadays—that BPDCN patients who get a myeloablative (full destruction of the marrow) transplant have as much a chance of survial as AML patients. This survival rate is not great, but it is possible. The better your attitude, your health, and the younger you are, the better your chances.
1. "Blastic plasmacytoid dendritic cell neoplasm: clinical features in 90 patients": "allogeneic bone marrow transplantation should be considered immediately, as it is currently the only option associated with long-term survival."
2. "Unrelated SCT induces long-term remission in patients with blastic plasmacytoid dendritic cell neoplasm": "We evaluated five patients allografted as consolidative treatment with an unrelated donor in first or subsequent remission. Four patients received a reduced intensity-conditioning regimen because of age or co-morbidities. As the stem cell sources, two umbilical cord blood-(UCB), two PBSC- and one BM graft were used. No GVHD was observed in the patients who received a UCB graft. However, both developed a post-transplant-associated lymphoproliferative disease. So far, only one patient has experienced relapse and was consecutively treated by escalated donor lymphocyte infusions (DLI). A potent graft-versus-leukaemia (GVL) effect was induced leading to a 17-month-long CR. Four patients are still in ongoing CR with median disease-free and overall survivals of 17 and 21 months. Thus, allogeneic SCT in BPDCN offers a potential curative option for patients with a compatible donor. UCB is an attractive alternative as a stem cell source. For relapsing patients, DLI can exert a powerful GVL effect."
Interpretation: 5 people received marrow transplants from compatible donors, but not from themselves (autologous transplant). Four were stem cell transplants (see "Bone Marrow Transplant" on tab above), two of those from umbilical cords. One was actual marrow pulled from a donor. (For our purposes, not being oncologists, we should consider a stem cell and a marrow transplant as just two methods of transplanting the marrow.)
3. Blastic plasmacytoid dendritic cell neoplasm: is transplantation the treatment of choice?: This study was the most cited one when I was diagnosed in 2011. It gives results for everyone they could find up to 2009. It's the study that told me that one person survived over 5 years without a marrow transplant, but that I had a 25-50% chance with a marrow transplant.
4. "Stem cell transplantation can provide durable disease control in blastic plasmacytoid dendritic cell neoplasm: a retrospective study from the European Group for Blood and Marrow Transplantation": "We conclude that high-dose therapy followed by allo-SCT from related or unrelated donors can provide durable remission even in elderly patients with BPDCN."
What Are the Symptoms
BPDCN used to be called NK-Cell Lymphoma (or something close to that). The difference between a lymphoma and leukemia is that leukemia results in cancer cells in the marrow and blood. Lymphoma is a blood cancer, too, but it produces tumors, usually associated with the lymph system (which includes the spleen).
Skin lesions—tumors and colored spots—are often the first evidence of BPDCN, so it was originally regarded as a lymphoma. Left alone, though, it almost always progresses into an aggressive leukemia. Chemotherapy is relatively successful at putting it into remission, but it always, except in one known case, relapses without a marrow transplant.
When I was diagnosed, I had two lesions on my back. They were the size and shape of a large cockroach, almost two inches long and about an inch wide. They stood up from the surface of my back about a quarter-inch. Over the next three weeks, as I awaited enough accurate diagnosis to begin chemotherapy, I started two more small tumors. One was inside my lower lip, so it produced no discoloration of the skin. The other was on my collarbone, and it was blue like the first two on my back had begun.
The two lesions on my back grew into a circle, and one grew so fast it tore the skin. They turned from blue to a reddish-purple.
|The dark spot on the upper tumor is where my skin tore from rapid growth.|
The day they put me in the hospital, laying on my back on the bed hurt because of pressure on the lesions. The day after chemo began, I could feel the lesions laying on my back, but it was comfortable. In four days, they had shrunk to flat, brown stains on my back, like a birthmark. The scab from the torn skin hung around a couple weeks, drawing doctor's comments every day about how unusual it looked.
I was not diagnosed by my lesions, however. They started as one tiny bump on my back that I thought was a mosquito bite, in February or March (2011). I didn't have them looked at by anyone till April, and that was a former nurse and a current emergency room nurse. The former nurse told me to have them checked, but thought they were just cysts. Three weeks later they were bigger and no longer smooth. The current nurse looked at it, and he sucked his breath in sharply. Then he asked, "Does cancer run in your family?"
I made an appointment shortly afterward.
In May, I could tell that my stamina was decreasing and pretty rapidly at that. In October of 2010, even though I was 49 years old, I had run 2 miles, all uphill, on a visit to California. In June, I ran a half-mile on a treadmill at a slow pace, and my forearms started to go numb and my legs hurt.
The doctor diagnosed me on a slightly low red blood count, a high white blood cell count, and deformed blood cells he could see under a microscope. He also noticed an absence of neutrophils, a tiny white blood cell that fights bacteria.
On June 21, my "hematocrit," related to red blood cell count, was 40%. Average for an adult male is about 48%. On July 12 or 13, just 3 weeks later, I was down to 25%. At somewhere between 15% and 18% I would die. They gave me blood on July 13 to prevent that.
One other symptom is a swollen spleen. A good doctor should be able to diagnose this and also swollen lymph glands by touch. My spleen was so swollen that I couldn't lean toward the left side. I could feel the pressure on the left side of my rib cage. The oncologist from the west clinic had no problem confirming that my spleen was swollen by feel.
Neither feel nor a CT scan showed swollen lymph glands, but often that precedes leukemia (blood) symptoms in BPDCN patients. Some BPDCN patients never show leukemia symptoms. Most have skin lesions.
Plasmacytoid dendritic cells come in two types, one of which dwells in the skin. (I'm sure that "dwells" is not proper medical terminology.) Apprently, that's the type that goes bad in BPDCN patients because lesions/tumors are in the skin.
Where Should I Seek Treatment?
Once you are diagnosed with a blood cancer, or the possibility of a blood cancer, you should be referred to an oncologist. That oncologist should give you a bone marrow biopsy. This is very much not the same as a transplant. They will pull some marrow from your hip, if you still have enough to pull, and send it to a pathology lab. Any oncology clinic should be able to do this.
Do not stop there, however. If you are diagnosed with BPDCN, you need to go to a good cancer center, such as Vanderbilt Medical Center in Tennessee, MD Anderson in Texas, City of Hope in southern California, or John Hopkins, which I believe is in Maryland. There may be a few others. Survival rates for hospitals that do a lot of bone marrow transplants are higher than in hospitals that do only 5 or 10 per year.
West Clinic in Memphis, which specializes in cancer and performes marrow transplants, was kind enough to tell me that I would do better at Vanderbilt in Nashville, especially because my leukemia was so unusual. At that point, a lab in California officially diagnosed me with BPDCN, though Vanderbilt later refused to confirm it, leaving me with a diagnosis of "Acute Undifferentiated Leukemia," which meant, for my purposes, "We don't know what it is except a very agressive blood cancer closely related to BPDCN."
Where Should I Seek Financial Help?
Try the Leukemia and Lymphoma Society, the American Cancer Society, United Cancer Advocacy Action Network, BMT Info, and your local Medicaid office, which is probably the same as your local welfare office.
If you have health insurance, see them first, of course.
Also, contact the social worker assigned to your oncology unit. I believe every major oncological hospital has one. Vanderbilt Cancer Center had two. Just ask your doctor, or better yet, your head nurse.
Can I Do Anything in Advance
Don't waste your time on natural remedies except for immediately improving your nutrition and exercise. Those of us who have had BPDCN and AML have tried everything. I juiced up to my first chemo and in between all my others. Several people recommended Gerson Therapy to me, but their web site says they know their therapy does not work on acute leukemia. ("There are certain types of cancer that do not respond well or do not respond at all. These include acute leukemia, pancreatic cancer after treatment with chemotherapy, and brain cancers other than an early stage astrocytoma. We have little or no experience with uncommon conditions of a congenital or genetic origin and would not expect the Therapy to reverse these conditions although overall health may be enhanced.")
I met one guy at a bone marrow transplant conference who claimed to have been healed of all GVH (Graft-versus-Host, which occurs only in those who have had an allogeneic marrow transplant) by ionized water. He said he had had seven cancers. I took him for a kook and a liar (just being honest). Here is an "unbiased" article. Here are studies that show ionized water helps with metabolic acidosis in dogs,(buildup of acid in the blood) and fetus and baby development during late pregnancy and lactation.
So maybe ionized water really will help with GVH, though I would never trust the guy who told me about it. Nonetheless, it's not going to heal your BPDCN in the few months or weeks you have to get it treated. Feel free to try it, but don't forego your chemo and transplant.
However, what you can do is be in the best shape possible to endure the chemo. Improve your diet, and as hard as it might be, start exercising. If walking is all you can do, then walk. They're going to press you to walk while you're in the hospital or getting chemo outpatient, anyway. Get in shape as best you can.
Simply put, eat right and exercise, even if it's only for 2 or 3 weeks before chemo starts, then continue during chemo.
Get a Great Attitude
Do anything you can to improve your attitude. Attitude is everything. Get saved, get positive, read self-help books. Do anything you can to go into your treatment with happiness, faith, and confidence. Fight. Be brave. All your nurses will tell you that they find a big difference between the survival rate of cheerful fighters and that of surly people feeling sorry for themselves.
You have every right to feel sorry for yourself, but if you do, you will probably die.