PRIMARY BONE NON-HODGKIN’SLYMPHOMA

PRIMARY BONE NON-HODGKIN’SLYMPHOMA

Large B Cell Primary Bone Non-Hodgkin’s Lymphoma – that was my diagnosis. I thought there was just one Non-Hodgkin’s Lymphoma – how wrong can you be. Someone told me there are 36 variations of NHL, and yesterday I read that there are 72 – so I don’t know how many there are to be honest. Perhaps this should be a new section within the Furry Monkey….. how many variations are there really?

After my diagnosis, I was told that Primary Bone NHL is very rare. I’ve since checked it out and it appears to only happen in 1% to 3% of people diagnosed with NHL. So this is indeed, rare. Trust me to have an unusual cancer, as John said “you couldn’t have a normal one could you?!”.

I’ve tried to find out more about it, but information is limited. I’ve put the following information together from various sources. I hope that you find it useful.

Find out all about Primary Bone Cancer
Normal Bone Tissue
What is primary bone cancer?
What is Primary non-Hodgkin’s Lymphoma of Bone?
So what is secondary bone cancer?
What’s the difference between primary and secondary bone cancer?
What symptoms can you get with bone cancer?
How are bone tumours diagnosed?
What bones can be affected by cancer?
What treatment is available for bone cancer?
What is the chance of recovery?

What is normal bone tissue?
Bone is the supporting framework of the body. Most bones are hollow. The outer part of bones consists of fibrous tissue called matrix onto which calcium salts are deposited. At each end of the bone is a zone of cartilage, a softer form of bone-like tissue. Cartilage consists of a fibrous tissue matrix mixed with a gel-like substance. Unlike bone, cartilage does not contain calcium. Cartilage acts as a cushion between bones and, together with ligaments and some other tissues, forms the joints between bones. The bone itself is very hard and strong. Some bone is able to support as much as 12,000 pounds per square inch. It takes as much as 1200 to 1800 pounds of pressure to break a femur (thigh bone).

The outside of the bone is covered with a layer of fibrous tissue called periosteum. The bone itself contains two kinds of cells. The osteoblast is the cell responsible for forming bone and the osteoclast is the cell responsible for dissolving bone. Although bone looks to be a very unchanging organ, the truth is that it is very active. New bone is constantly forming, and old bone dissolving.

Bone marrow is the soft tissue inside the hollow bones. The marrow of some bones consists only of fatty tissue. The marrow of other bones is a mixture of fat cells and hematopoietic (blood- forming) cells. These blood-forming cells produce red blood cells, white blood cells, and blood platelets. There are some other cells in the marrow such as plasma cells, fibroblasts, and reticuloendothelial cells.

What is primary bone cancer?
Primary bone cancer refers to cancers which start in the bone. These cancers are totally different to secondary cancers which start in other parts of the body and then spread to the bones. Bone cancers are tumours which have the potential to spread to other parts of the body.

These are different to benign (non-cancerous) bone tumours which do not spread. Benign bone tumours are more common than malignant (cancerous) bone tumours.

There are several different types of bone tumors. Their names are based on the area of bone or surrounding tissue that is affected, and the kind of cells forming the tumor. Some primary bone tumors are benign (not cancerous) and others are malignant (cancerous). Most bone cancers are called sarcomas. Sarcomas are cancers that mostly develop from bone, cartilage, muscle, fibrous tissue, fatty tissue, or nerve tissue.

What is Primary non-Hodgkin’s Lymphoma of Bone?
Primary non-Hodgkin’s lymphoma of bone (PLB) is a rare type of cancer starting in bone, it accounts for about 1% to 3% (that I’ve found so far!) of all primary bone tumours. This was my diagnosis for NHL.

Lymphomas can arise from almost any lymphatic tissue (lymph nodes, spleen, thymus, mucosa associated lymphoid tissue (MALT)). It is hard to believe but the bones are an important part of the lymphatic process: the “B” in white b-cell is for cell maturation in the bone marrow.

Non-Hodgkin’s lymphoma (NHL) usually starts in the lymph nodes and lymph glands (part of the immune system). PLB, however, starts in the bone. This is distinct from NHL which started in the lymph notes and then spread to the bones (bone metastases).

The peak age of people diagnosed with PLB is in the 50-60 yr age group, the disease is slightly more common in men than in women. Symptoms are usually bone pain and sometimes swelling. The majority of people with PLB are diagnosed with a single localised tumour. PLB has higher survival rates compared to other types of Non-Hodgkin’s Lymphoma.

So what is secondary bone cancer?
Secondary bone cancer is where malignant cells have spread to the bones from other parts of the body. This is totally different to cancers that have started in the bones (primary bone cancer). Virtually all types of cancer can spread to bone. Bone metastases are particularly common in people with breast, lung or prostate cancer. Bone metastases are usually multiple, and they can cause pain and can lead to other symptoms such as hypercalcemia (abnormally high levels of calcium in the blood).

What’s the difference between primary and secondary bone cancer?
There are really two main types of bone cancer. Primary bone cancer is a cancer that starts in the bone. It’s less common than secondary bone cancer, which starts somewhere else in the body and then spreads to the bone. Secondary bone cancer is more common.

Any type of cancer can spread to the bone but bone metastases (spreading of cancerous cells to the bone) is most common among persons with breast, lung, or prostate cancer.

What symptoms can you get with bone cancer?
The symptoms of bone cancer vary from person to person and depend on the location and size of the tumour. Two people with the same type of bone cancer can have very different symptoms.

The most common symptoms of cancer are pain and swelling or tenderness in the affected area. You may see or feel a hard lump on the surface of the affected bone. Sometimes bone cancer can interfere with movement and can weaken the bones, occasionally leading to a fracture (this is what they first suspected I had in my leg). Other symptoms of cancer may include tiredness, fever, weight loss, and anaemia.

With NHL some of the more well known symptoms are:

  • Chills
  • Painless swelling of lymph nodes
  • Fever
  • Night sweats
  • Unexplained weight loss
  • Lack of energy
  • Itching

None of these symptoms is a sure sign of cancer; if you suspect you have a health problem please consult your doctor.

How are bone tumours diagnosed?
If a bone tumour is suspected the doctor will do a complete medical examination. This may include a blood test as bone tumours can be associated with increased levels of certain proteins in the blood. The doctor may also recommend X-rays and other scans of the bone(s).

If the results of the x-rays and scans suggest that a tumour may be present then a biopsy (removal of a sample of tissue) will be performed. A pathologist will then examine the cells to determine whether it is cancerous, and if so what type of cancer it is that you have.

What bones can be affected by cancer?
In total there are over 200 bones in the human body, any of these can be affected by cancer. However, certain types of bone cancer are more common in specific bones.

Osteosarcoma is most commonly found in the bones around the knee. Ewing’s sarcoma is more common in the upper leg, pelvis and other bones of the trunk. The pelvis is the most frequent location for chondrosarcoma. My NHL is in both my left and right leg. The tibia (below the knee) in the left leg and above the knee in the right leg.

Nevertheless, it is possible for these cancers to affect any bone in the body.

What is the treatment for bone cancer?
Everyone’s treatment depends on their own cancer. For example the type of cancer it is, whether it has spread or not, and the size and location of the main (primary) tumour. Treatment of bone cancers is complex and involves a team of different specialists usually within an institution that is experienced in treating these types of cancers.

There are 3 main types of therapy used to treat bone cancers:

  • surgery (to take out the tumour in an operation)
  • radiotherapy (using high-dose x-rays to kill cancer cells)
  • chemotherapy (using drugs to kill cancer cells)

Surgery is often used to remove the primary tumour. For tumours of the arms and legs an amputation of the limb is sometimes necessary, however, limb-sparing surgery may be possible in many cases where only the cancerous part of the bone is removed and it is replaced by a bone graft or metal prosthesis.

Radiotherapy may also be given as well as or instead of surgery to destroy the cancer cells. Chemotherapy may also be given to kill malignant cells that may be circulating around the body.

It was decided I was to have the first option, surgery, to remove the tumour in my leg and then replace part of my tibia and my knee with metal implants. However at my next appointment it was then decided to go with the chemotherapy/radiotherapy route instead and see what happens. Thankfully no surgery has been required – although they have told me not to rule this out in the future.

What is the chance of recovery?
Overall, the chance of recovery (prognosis) for bone cancers has improved significantly since the development of modern chemotherapy.

However the chance of recovery will depend on a variety of influences; if the cancer has spread, the type of bone cancer, the size of the tumour, location, the person’s general health and other individual factors. Also important is how much of the main tumour can be removed/destroyed by surgery and/or radiotherapy and chemotherapy, and how the tumour responds to chemotherapy.

Your consultant should be able to give you a rough idea of the prognosis for your type of cancer.

As I said earlier, all diagnosis and future treatment of any cancer, not just Primary Bone NHL is determined by your consultant and/or doctor. They will have all the results of your tests and scans and will be able to advise you.

However please note that not all consultants are keen on answering questions as I have found!

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