Originally from: My Life With Lymphedema Blog Spot
OK...so a dear friend wrote me and said, "the blog needs to be more about the person then lymphedema." LOL...so I guess that is what a blog is for
Clinical Considerations for the Diagnosis and Management of Lymphoma with Primary Lymphedema
The key to understanding lymphoma as a secondary condition to primary lymphedema is in the pathophysiology of the lymph system.
In the situation of primary lymphedema, the lymph system is either incompletely formed or malformed. This means that the spread of and the expression of lymphoma (course of the disease) is going to be different then in an individual with a "normal" lymph system.
The reason is simple. With the lymphatic flow constricted, the malignancy will have a more difficult time spreading to external nodal areas. The incomplete lymph system will act as a "damn" actually helping to contain the lymphoma.
Beause of either the hypoplaysia or hyperplaysia of the lymphatics, it is going to be more difficult to obtain an accurate diagnosis of a lymphtic cancer.
The nodes of a person with lymphedema may already be smaller then normal. Therefore, if there is limited nodal enlargment (or even if there is none) you can not assume no malignancy is present.
Furthermore, I have yet to experience really significant lymph node enlargement. But, what I have experienced are nodes that are only slightly larger then "normal" yet have turned hard and rubbery.
Standard radiological tests must be viewed carefully in using them to chart lymphatic cancer with lymphedema. MRI's and CAT's are of limited use as the "normal" size node they are picking up, may infact be malignant.
Case in point: When the small needle biopsy of my right inguinal lymph node was performed, the doctor did an ultrasound first. That node looked perfectly normal to the ultrasound. The biopsy came back positive.
The use of a PET scan in the staging and diagnosis must be viewed with skepticism. There are important reasons for this. The principle behind the PET is quite simple. Tumor (malignancies) will have a higher metabolic rate then the surrounding nonmalignant tissues. Therefore, the malignancies will have a higher "uptake" of the contrast used.
Because of the dysfuntion of the lymph system, it is going to be more difficult for the contrast to make its way through the body and be absorbed. This will effect the diagnostic effectiveness.
Unfortunately too, there is also no standard uptake value for tissue effected by lymphedema. Therefore, it is going to be difficult to ascertain whether a SUV number indicates a malignancy or not.
On the opposite side, lymphedema patients are faced with constant inflammation and/or chronic low grade infection. Both of these can give a false positive to a PET scan.
After the diagnosis of a lymphatic cancer is achieved, the treating physician must carefully monitor changes in the complications experienced by the patient. New complications and changes must not automatically be assumed that they are caused by lymphedema.
Case in point : Before the spread and/or infilitration of lymphoma throughout my system, I did not have lung fluid. Two years ago, I began having fluid accumulate in my right lung. This year, the left lung has also been extensively involved.
Also, my lymphedema has been consistent for decades. Also, during the past two years, I have experienced swelling of my left arm for the first time.
It is important to understand that those of use with a dysfunctional lymph system walk a fine line. In my situation, with an already at risk lymph system, the lymphoma spreading through the chest was enough to "overload" the impaired system resulting in both pleural effusions and swelling of the left arm.
Perhaps the most important point to make is for the physician to "listen" to the patient. Individuals like myself have lived with lymphedema for decades - most for years.
We know how lymphedema effects our bodies, what is (normal) for ourselves and what should or should not be happening.
Listen...care about...communicate with your patient.
Lymphoma and Lymphedema - an Update
It has been awhile since I put an update on my lymphedema and lymphoma so thought I would sharewas has been happening.
I went through four weeks of Rituxan chemo in August/September. I experienced immediate results and even a tumor of the lower back calf actually shrank and started to look like it was caving in on itself.
The results were shortlived however, and the tumor rosbustly sprouted back to life...seemingly to grow actually faster then it had before.
So, I was off to have a "punch" biopsy. The results took forever and getting an actual diagnosis was even more complicated. This was a result of possible inflammation and the fact that this tumor had already been hit with chemo.
But, what it showed was more then interesting and has caused more questions then it provided answers.
The B cell lymphoma is still definitely there and apparently active. But the results also showed that about 40% of the cell population of the tumor was comprised of "T" cells which also came back with a positive stain for CD3. Is this because of possible inflammation...or are we witnessing the birth a yet another lymphoma? Possible T cell lymphoma? We honestly can't say and don't know.
Another very interesting report was on the skin itself. My lymphedema is caused by "hypoplasia" of the lymphatics. This means the lymphatic system is constricted and/or missing important parts. Tests when I was a teen showed that I am missing significant lymph nodes in the inguinal regions.
April 30, 2006 Part Three
I have continued the Rituxan treatments, and hopefully that is helping blunt and/or remove the lymphoma. This will be continued every three weeks for the next two years.
There has however, been a dramatic change in the lymphedema. For the last couple months, I have experienced significant over-all body lymphedema.
This includes incredible difficulty in breathing, significant new swelling now in both arms, abdomen, chest and even back.
For many years, I tried to get my former oncologist to understand that B-cell lymphoma with hereditary lymphedema would not follow the same progression path and lymphoma by itself.
As all this new lymphedema has occured, I have wondered how much of it is lymphoma...how much is just the lymphedema.
I ran accross quite by accident today, the first clinical report I have been able to locate on B-cell lymphoma and its overall effect on the lymph system itself. For me it is stunning, as it is verifiable proof of what I have been saying for years.
Density of intranodal lymphatics and VEGF-C expression in B-cell lymphoma and reactive lymph nodes.
Department of Haematology, Blood Neoplasms and Bone Marrow Transplantation, Medical University, Wroclaw, Poland.
Lymphatic vasculature in solid tumors may serve as the pathway for metastatic spread of the cancer to the regional lymph nodes and to distant organs. Controversy still exists whether tumors metastasize through existing lymphatics or through newly formed vessels (lymphangiogenesis). The role of lymphangiogenesis in lymphoma spread and proliferation is not clearly established.
VEGF-C is the most potent inducer of lymphangiogenesis. LYVE-1 was shown to be a specific marker for lymphatic vessels in normal and tumor tissue. The aim of the present study was the evaluation of lymph node LYVE-1-positive lymphatic sinus density (LSD) and VEGF-C expression in patients with non-Hodgkin's lymphoma (nHL) and in reactive lymph nodes. Sixty paraffin-embedded lymph nodes from newly diagnosed patients with B-cell nHL were evaluated. Twelve lymph node biopsy specimens from adult patients with reactive lymphonodulitis were used as controls. Sections of lymph nodes were stained immunohistochemically for LYVE-1 and VEGF-C.
In all groups we demonstrated a strong positive correlation between VEGF-C and LYVE-1-expression (p = 0.0001). Higher LSD in reactive lymph nodes as compared to those of nHL patients suggests that lymphoma proliferation leads to the destruction of the existing lymphatics rather than to lymphangiogenesis within lymph nodes. NHL are not associated with increased expression of VEGF-C nor increased LYVE-1-positive lymphatic sinuses density within lymph nodes.
It has been a very long time since I posted on how my life is with lymphedema and lymphoma, so I thought I would put in a brief update.
Something started going terribly wrong with my body in early 2006 and I began to experience total body lymphedema. My abdomen and chest filled up with fluids and I was not able to stop or prevent it from becoming worse.
It was truely one of the most horrible experiences of my life as I became more and more incapacitated and the pain associated with it was overwhelming, both physically and psychologically.
I became unable to do even the most simple of tasks like tie my shoe and even eventually could no longer take care of my legs by putting lotion on them and bandage wrapping them.
By the time of the National Lymphedema Network's conference in very early November, I was barely able to get around with a cane. At the conference, I was confined to a wheelchair for a large portion of the conference. I honestly could not have made it through the conference without the help of Joan White and Cheri Hoskins who were always there to help and to push me from event to event. A therapist name Carmelita Rifkin also came each dayto my room to wrap and care for my legs. All three were truely angels of kindness and I will always have a deep deep appreciation for all they did.
The conference ended and I returned to work, for only a week. By November 12th, I was so full of fluids that I could breathe only while standing up. My son, Patrick, took me to the emergency room at Gwinnett Medical Center and I was subsequently admitted as an inpatient.
I was in that hospital from November 12, 2006 to December 8, 2006 when I was transferred by ambulance to the Lakeshore Rehabilitation Hospital in Birmingham, Alabama to be under he care of Dr. Paula Stewart.
All in all, the doctors were able to remove around sixty pounds of fluids of my chest and abdomen. During the first week at Gwinnett, they also drained two and a half quarts of fluid from the right lung. Interestingly, the fluid has always previously been a golden amber color, this time it was pure chylous...milky white in color. On December 22, 2006 I was finally released from Lakeshore and came home.
Then, in the afternoon of December 27th, I began to experience horrendous pain in my back between the left shoulder blade and spine. Again, i was taken to Gwinnett and admitted with a pnuemothorax. They again drained my right lung. In only five weeks, two quarts of fluid had returned to the lung.
I was released on December 31st and finally returned to work on January 4, 2007.
But, something had to be done about the lung fluid as you can not safely keep going back time after time to drain the lung, so a pulmonary doctor and a thoracic surgeon was called in.
This coming Monday, Januar 15th, I will be admitted yet again to Gwinnett and undergo a surgical procedure. Several small incisions will be made and the area between the lung lining and the rib lining will be filled with talcum powder in hopes that it will effectively block off the cavity from further fluid accumulation.
While the doctor has said there may be only a 50-50 chance of success due to the lymphedema, I feel it is a chance I must take. Each month now, it seems that right lung fills up with a couple liters of fluid. There are only so many times that you can drain a lung before complications set in.
The worse part and perhaps the most painful is this tube that will be sticking out of my side for a few days. While he is in there, a small camera will be inserted and some biopsies of the pleura will be taken. If all goes well, he will drain the left lung too, as it shows about 1 1/2 liters of fluid.
The bad thing is, is that no one really has any clear explanation of what triggered all these events. It was also discovered that my blood protein level had dropped to a dismal 1.5, which is far far short of the ideal 4.5-5.5 level.
Dr. Stewart has one theory that ties in the drop in the proein level, tied in with the lymphedema to cause all the swelling and fluid collection.
The other theory involves the lymphoma. My incredible oncologist, Dr. Stephen Szabo would describe the lymphoma as "not in remission but contained." So somehow perhaps everything just went together to cause this disaster.
I'm just so relieved though that thus far, the massive swelling has not returned and that I am once again able to do so many things...especially play with my new grandson Connor, who was born on October 15, 2006.
Life is meant to be a celebration of the things we can do, not a requiem for this things we can not do. The great Native American chief Tecumseh once said "When you arise in the morning, give thanks for the morning light, for your life and strength. Give thanks for your food and the joy of living. If you see no reason for giving thanks, the fault lives in yourself"