Lymph nodes are a normal part of the immune system. Lymph nodes are small, typically pea sized glands that are present in all parts of the body. There are approximately 600 lymph nodes in the body with only the lymph glands in the neck, arm pits and in the groin that can be usually felt.

The majority of causes of lymphadenopathy are not serious and are usually caused by infections. Viral infections are often a cause for lymph nodes increasing in size. Lymph nodes that increase in size of number because of a viral infection usually return to normal size in week or two.

There are other more serious causes of lymph nodes increasing in size or number. Some cancers can begin in lymph glands or spread to lymph glands from other sites in the body. Cancer presenting a lymph gland enlargement is fortunately uncommon with one study with over 2500 patients who went to their doctor with large lymph glands showing only 1% of patients having cancer as the cause for the large lymph glands. Cancer is even less commonly diagnosed in younger patients presenting with lymph gland enlargement.

It will be important for you to see your doctor and explore if you have any other symptoms that may suggest a cause for your lymph glands increasing in size (e.g. recent cold or infection, any other symptoms such as loss of weight). Your doctor will then exam the area to find out what size the lymph glands are, if they are painful and what the consistency of the lymph glands are (e.g. soft, hard, can be moved under the skin etc). Your doctor will also look to see if any other changes associated with the lymph glands enlarging (e.g. skin changes, areas of local infection).

Sometimes a period of waiting and watching for the lymph glands to return to normal size is necessary before doing any tests.

Other times your doctor may arrange blood tests or scanning tests to get more information about the cause for the lymph glands to be increased in size. Blood tests looking for recent viral infections may be helpful and an ultrasound of the lymph node may be helpful in making sure the large lymph gland is not caused by anything serious.

In a few cases, a biopsy of the gland may be needed if the cause for the lymph gland increasing in size is no known. This is usually done by a surgeon who will remove the lymph gland and send it to the laboratory for testing. This type of surgery is usually very short and safe but is only needed occasionally to sort out why lymph glands are enlarged

HS is relatively common – around 1 person in every 5000 people has HS (around 800 people in Melbourne; population 4 million).

There are three main common problems associated with having HS:

There is another uncommon potential problem for people who have Hereditary Spherocytosis associated with a viral infection called “Slapped cheek”. Slapped cheek is caused by a virus called Parvovirus which can infect bone marrow cells and put the red cell producing cells “to sleep”. Because patients with HS need the bone marrow to be rapidly replacing the fragile red cells, this infection can cause a severe anaemia; patients can become very pale and sometimes even require blood transfusions. Fortunately this problem is uncommon.

HS is now diagnosed with a simple blood test. It is also important for a doctor to examine patients with HS to see if they are jaundiced or have an increase in the size of the spleen.

Most patients with HS do not need to do much about their condition at all. The bone marrow has the capacity to increase the number of red cells it produces many fold and is able to keep up replacing the fragile cells.

A few things may be helpful:

Removing the spleen (splenectomy) has been used a lot in the past in the treatment and management of patients with HS; it stops the red cells from breaking down and solves the problems of HS.

Removing the spleen has problems however – the spleen is an important organ in the immune system and patients who do not have a spleen may be prone to getting serious infections. This risk is higher in younger children but may be less than previously expected because of newer vaccinations. It is now generally recommended to avoid splenectomy in children younger than 6 years and to ensure that children who may need a splenectomy have all the appropriate vaccinations.

Another approach for some of these young patients is to remove only a small part of the spleen – partial splenectomy. This is done in a few hospitals and there is some experience to say this may be very helpful for some patients.