Nodular lymphocyte-predominant Hodgkin lymphomacancers of the lymphatic system (NLPHL) is a rare form of Hodgkin lymphoma that is characterised by variants of Reed-Sternberg cellsthe basic structural and functional unit of all living things known as ‘popcorn’ cells because of their appearance. It develops slowly and is often diagnosed earlier than the classical subtypes of Hodgkin lymphoma.
The lymphatic systema network of tissues and organs that help our bodies fight infection and disease is a network of tissues and organs that help our bodies fight infectiona condition where harmful pathogens, such as bacteria, viruses or parasites, have entered the body and disease. It is composed of lymph vesselsa network/chain of thin tubes that moves lymph fluid around the body, lymph fluida clear watery fluid that carries nutrients around the body and removes unwanted bacteria/viruses, and lymph nodessmall bean-shaped structures that filters harmful substances from lymph fluid/glands. Some of the most well-known lymph tissues include the bone marrowsoft, spongy tissue found in bones that makes blood cells, the spleen, and the tonsils.
NLPHL is more common in males, and tends to be diagnosed between the ages of 30-50. However, anyone can develop this disease.
Treatment
If NLPHL is detected, it will be staged and graded based on size, metastasiswhen the cancer has spread to other parts of the body, also known as mets, and how the cancera disease where abnormal cells split without control and spread to other nearby body tissue and/or organs cells look under the microscope. Stagingthe process of determining how big the cancer is, where it started and if it has spread to other areas and grading helps your doctors determine the best treatment for you.
Cancers can be staged using the TNM staging system:
- T (tumoura tissue mass that forms from groups of unhealthy cells) indicates the size and depth of the tumour.
- N (nodea small lump or mass of tissue in your body) indicates whether the cancer has spread to nearby lymph nodes.
- M (metastasis) indicates whether the cancer has spread to other parts of the body.
This system can also be used in combination with a numerical value, from stage 0-IV:
- Stage 0: this stage describes cancer cells in the place of origin (or ‘in situ’) that have not spread to nearby tissuea group of cells that work together to perform a function.
- Stage I: cancer cells have begun to spread to nearby tissue. It is not deeply embedded into nearby tissue and had not spread to lymph nodes. This stage is also known as early-stage cancer.
- Stage II: cancer cells have grown deeper into nearby tissue. Lymph nodes may or may not be affected. This is also known as localisedaffecting only one area of body cancer.
- Stage III: the cancer has become larger and has grown deeper into nearby tissue. Lymph nodes are generally affected at this stage. This is also known as localised cancer.
- Stage IV: the cancer has spread to other tissues and organs in the body. This is also known as advancedat a late stage, far along or metastatic cancer.
Cancers can also be graded based on the rate of growth and how likely they are to spread:
- Gradea description of how abnormal cancer cells and tissue look under a microscope when compared to healthy cells I: cancer cells present as slightly abnormal and are usually slow growing. This is also known as a low-grade tumour.
- Grade II: cancer cells present as abnormal and grow faster than grade-I tumours. This is also known as an intermediate-grade tumour.
- Grade III: cancer cells present as very abnormal and grow quickly. This is also known as a high-grade tumour.
Once your tumour has been staged and graded, your doctor may recommend genetic testinga procedure that analyses DNA to identify changes in genes, chromosomes and proteins, which can be used to analyse tumour DNA to help determine which treatment has the greatest chance of success, which analyses your tumour DNA and can help determine which treatment has the greatest chance of success. They will then discuss the most appropriate treatment option for you.
Treatment is dependent on several factors, including age, stage of disease and overall health. The types of treatments generally don’t vary between adults and children.
Treatment options for patients with NLPHL may include:
- Chemotherapya cancer treatment that uses drugs to kill or slow the growth of cancer cells, while minimising damage to healthy cells.
- Radiation therapya treatment that uses controlled doses of radiation to damage or kill cancer cells.
- Surgerytreatment involving removal of cancerous tissue and/or tumours and a margin of healthy tissue around it to reduce recurrence, most commonly a lymphadenectomysurgical removal of lymph node(s).
- Watch and waitthe close monitoring of a cancer without giving treatment until symptoms appear or worsen.
- Clinical trialsresearch studies performed to test new treatments, tests or procedures and evaluate their effectiveness on various diseases.
- Palliative carea variety of practices and exercises used to provide pain relief and improve quality of life without curing the disease.
Risk factors
While the exact cause of NLPHL remains unknown, the following factors might increase the riskthe possibility that something bad will happen of a person developing this type of cancer.
- Having been infected with HIV or EBV.
- Being immunocompromised.
- Family history of Hodgkin lymphoma.
- Exposure to chemical solvents, such as certain pesticides and fertilisers.
Not everyone with these risk factors will develop the disease, and some people who have the disease may have none of these risk factors. See your general practitioner (GP) if you are concerned.
Symptoms
The main symptom of NLPHL is a firm, painless lump I the neck, armpits and/or groinarea between the abdomen and thighs. People may also develop what is known as ‘B symptoms’, which are:
- Extreme night sweats.
- Persistent fevers.
- Unexplained weight loss.
Not everyone with the symptoms above will have cancer but see your general practitioner (GP) if you are concerned.
Diagnosis
If your doctor suspects you have Hodgkin lymphoma, they may order the following tests to confirm the diagnosisthe process of identifying a disease based on signs and symptoms, patient history and medical test results and refer you to a specialist for treatment:
- Physical examinationan examination of your current symptoms, affected area(s) and overall medical history.
- Blood teststesting done to measure the levels of certain substances in the blood.
- Imagingtests that create detailed images of areas inside the body tests (if the cancer is thought to have spread beyond bloodthe red bodily fluid that transports oxygen and other nutrients around the body and bone marrow), potentially including:
- MRI (magnetic resonance imaging)a type of medical imaging that uses radiowaves, a strong magnet and computer technology to create detailed images of the body.
- CT (computed tomography) scana type of medical imaging that uses x-rays and computer technology to create detailed images of the body.
- PET (positron emission tomography) scana type of medical imaging that uses radioactive tracers to create detailed images of the body.
- X-raya type of medical imaging that uses x-ray beams to create detailed images of the body .
- Bone marrow aspirationa procedure that involves inserting a needle into the hipbone (or the breastbone in some cases) to remove samples of solid and liquid bone marrow..
- Biopsyremoval of a section of tissue to analyse for cancer cells.
