The information in this booklet was originally written by Drs Scott and Richards. It has been revised by Dr Paul Revell, Consultant Haematologist at Staffordshire General Hospital and a member of the Lymphoma Association Medical Advisory Panel.

This booklet aims to provide important information essential for those lymphoma patients who have had their spleen removed. Please telephone the Helpline if you would like clarification of any of the information here, or if you would like further information.

The spleen is an organ about the size of a large pear which lies just under the rib cage on the left hand side of your body. It has two major jobs, carried out by different working parts. The first is a very fine network of blood vessels which filter the blood. These blood vessels are lined with killer cells (called phagocytes or macrophages) which are able to eat large foreign particles in the blood. The spleen is responsible for clearing out debris such as old red blood cells. The second is special lymphoid tissue which makes specific immune or defensive responses.

Throughout life our immune systems develop antibodies and immunity to the infections and illnesses we encounter. Not having a spleen means that certain parts of the immune system no longer work as effectively, though many of the functions of the immune system carry on in the liver, bone marrow and in lymph glands all over the body. Without a spleen, we are less able to fight off certain rare but serious infections.

What happens with 'splenectomy' (removal of the spleen)?

In fact splenectomy is rather infrequently needed to help lymphoma patients nowadays, though it remains a good treatment in certain cases. When required, the procedure carries the usual risks of anaesthesia etc but is often straightforward with good recovery (many patients will go home in less than a week). There will be a scar in the upper abdomen which will usually fade. From the time the spleen has been removed, however, you are at risk from severe infections, but will be offered protection.

Infections involved

Without protection, there is a risk of harmful (and sometimes fatal) infections with Streptococcus pneumoniae (pnuemococcus), Haemophilus influenzae, Neisseria meningitidis (meningococcus) and malaria. Also tick-borne babesiosis, Capnocytophaga canimorsus from dog bites and, to a lesser extent, other streptococci, E.coli, pseudomonas and staphylococci. The risk, therefore, is of uncontrolled infection or 'overwhelming post splenectomy infection' (sometimes abbreviated to OPSI).

How high is the risk of infection?

Doctors do not know the exact magnitude of the risk of infection but it is about 12 times the normal incidence overall. This increases to 50-100 times for children and lymphoma patients, especially those with Hodgkin lymphoma (formerly Hodgkin’s disease). This may be because the disease itself leads to a general reduction in the immune system and chemotherapy adds to this. Although it improves with time, the risk of severe infection never returns to normal.

It is important to point out that, although what you have read so far may seem a little alarming, the risk overall is still low. The risks are likely to be even less if the protective measures listed below are followed.

Five important points to remember:

• Try to make sure that the fact that you have no spleen is stated prominently on the hospital and GP notes, and in the computer record if they have one.

• You should have one-shot vaccination of Pneumovax 2, Hib vaccine and Meningococcal A&C, unless you have had them already.

Five years is the earliest you should discuss revaccination with Pneumovax 2 (it may, in any case, not add extra protection).

The guidelines are not definite about Meningococcal A&C vaccine and is only suggested if you are going abroad. As you may not remember when organising a holiday, and since there have been cases with these strains in the UK, it is recommended that you have the vaccine anyway. It does not prevent you having any improved meningococcal vaccines at a later date - indeed your GP may suggest the new Meningococcal C vaccine instead (most children have already had this vaccine).

• Ideally you should be on Penicillin V 250mg twice a day for life.

The dose might be higher around the time of surgery. Most patients find a twice daily dose quite convenient. There are alternative strategies, which you can discuss with your doctor, if you are allergic to penicillin or know that you won’t get around to taking tablets regularly. The vaccines are good but do not protect against everything.

• You should take special precautions against malaria.

If you are travelling to a country where malaria is present you should employ all the physical anti-mosquito measures such as nets, 'plug in' devices and creams, as well as taking anti-malaria tablets. There are a number of other infections abroad which can pose a threat (eg babesiosis in the USA), so if you are unwell abroad you must let your doctor there know that you have no spleen.

• You should carry a card.

It is always worth mentioning to your medical attendants that you have no spleen, for example if you have had a dog-bite, or if you call out a doctor who does not know your medical history. The Lymphoma Association has a supply of the national splenectomy card with an accompanying leaflet. Most hospital haematology and oncology departments have them too.

Remember - the chance of serious problems after splenectomy are low - but a number of simple measures can make them very low indeed.