One of the many complications of CLL is an enlarged spleen; the technical word for it is “splenomegaly”. In simple terms, your spleen becomes enlarged when it is stuffed full of CLL cells and other cellular debris. So, if you are sporting a slightly pregnant look without actually being pregnant, you might want to talk to your doctor about the status of your spleen.
Most people go through their entire lives without ever thinking about their spleen. I am willing to bet majority of people do not know where in the body it is located or what the heck it does for a living. You are about to become one of the educated minority that knows all about splenic function! One of the better side effects of CLL is the terrific biology education you never thought you would get in your life!
Think of the spleen as a blood filtering organ and you won’t be far off the mark. It is about the size of your clenched fist and located just a tad lower than your rib cage, on the left hand side. Under normal circumstances it is not possible to feel your spleen by mere poking around, even in skinny people. But when it is engorged (either due to CLL or some other illness such as infection, liver disease etc) trained physicians can feel the tip of the spleen just below the rib cage by physical palpation. If your spleen can be felt by palpation, it is already enlarged beyond its normal size. A CAT scan will reveal exactly how enlarged it is and whether it needs to be treated.
Symptoms of an enlarged spleen
Most often you are not likely to feel any symptoms of spleen enlargement, at least during early stages of the process. But as it grows larger it may press against your stomach and that may cause you to feel full – even if you have just eaten only a small snack. I am told that patients with very large spleens walk / balance funny, not unlike pregnant women, and this may cause back pain. More important symptoms of an enlarged spleen are anemia and thrombocytopenia – low red blood cell counts and low platelet counts, respectively. These are the two symptoms that matter most to us as CLL patients and I will be focusing on them in the rest of this article. As you would guess, side effect of low red blood cell counts is deep fatigue. Red blood cells carry oxygen and with reduced oxygen carrying capacity fatigue follows soon after. Similarly, low platelet counts mean poorer blood clotting capability and you may find you are prone to bleed longer if you cut yourself accidentally.
What does a spleen do?
As I said before, your spleen acts like a spongy filter for your blood. As blood circulates through your body, some of the cells (red blood cells, platelets) get worn down and damaged. It is the function of the spleen to remove these damaged cells from circulation. The spleen also acts as a storage location for extra red blood cells and platelets newly minted by your bone marrow.
Unlike red blood cells and platelets which are manufactured only in your bone marrow, lymphocytes can also multiply in the lymph nodes and the spleen. In healthy individuals the spleen is important in putting out additional troops of lymphocytes (white blood cells) when an infection is detected. Hence, your spleen is an important part of the first line of defense in fighting invading pathogens. The spleen also serves to trap some bacteria.
An enlarged spleen affects each of these vital functions. For instance, as your spleen grows larger, like any clogged filter it begins to trap (“sequester”) normal red blood cells as well as abnormal / damaged ones, reducing the number of healthy cells available for circulation in your bloodstream. It also traps too many platelets. Eventually, excess blood cells and platelets trapped there can further clog your spleen, interfering with its normal functioning. An enlarged spleen may even outgrow its own blood supply, which can damage or destroy sections of the organ.
What causes splenomegaly?
A number of infections can cause spleen enlargement, quite apart from the CLL related issues we discussed above. For example:
- Mononucleosis and other viral infections can cause splenomegaly. Teenagers are typically more likely to have clinically significant EBV infection precipitating mononucleosis (“glandular fever” for the Brits in our membership) and therefore more likely to have splenomegaly for that reason.
- Bacterial infections – syphilis can do it. Endocarditis – an infection of your heart’s inner lining – can also cause spleen enlargement.
- Malaria (and other parasitic infections) are frequent culprits.
- Liver cirrhosis and other such diseases can spill over into the spleen as well.
- If you develop a blood clot in the veins servicing the liver or spleen, that can cause blood flow to back up and cause the spleen to swell.
- Blood cancers such as CLL, NHL and Hodgkin’s disease can cause splenomegaly.
- Certain metabolic disorders such as Gaucher’s disease and Niemann-Pick disease can also interfere with proper spleen function. People of Ashkenazi Jewish ancestry are thought to be more prone to these two metabolic disorders and therefore more at risk of splenomegaly for these reasons.
- People of African ethnicity are more prone to splenomegaly since it is frequent complication of sickle cell disease.
An enlarged spleen in a CLL patient can pose special problems. As the filtering capacity of the spleen gets compromised, perfectly good red blood cells and platelets can get trapped in there and you will see a sharp drop in these blood cell counts in your monthly CBC blood test. Even if your bone marrow is doing its job and turning out the required number of red blood cells and platelets, and there is no trace of autoimmune disease, the fact that your spleen is trapping these good cells can play havoc with your blood counts. And as you know by now, it is not a good idea to live long without healthy red blood cell or platelet counts.
Equally serious is the risk of a ruptured spleen. Even in healthy people it is possible to get spleens damaged, especially in car crashes and contact sports like football. Rupture is a much more likely possibility when your spleen is enlarged. A ruptured spleen can quickly become life-threatening by uncontrolled bleeding into your abdomen.
It is important to understand the reason for the enlarged spleen, and therefore target the treatment to correct the problem. If the cause for splenomegaly is an infection of some sort, then it is appropriate to treat the infection with appropriate antibiotic or anti viral drugs. But if the underlying problem is CLL, then it makes sense to treat the CLL itself with appropriate chemotherapy / chemoimmunotherapy. In many cases treating the spleen may take center stage if it is causing sharp drop in red blood cell counts and platelets.
Very often a swollen spleen makes things worse in cases of autoimmune diseases such as AIHA (autoimmune destruction of red blood cells) or ITP (autoimmune destruction of platelets) and it becomes impossible to stabilize blood counts without removing the spleen surgically. A Splenectomy (surgical removal of the spleen) is an option worth considering in critical cases of AIHA and ITP, and for many patients it may be the best option available.
I know, it is not fun to contemplate leaving behind a big chunk of your self behind on the operating table. But we must all learn to make the tough choices when we have to make them and not dither ourselves into a worse situation. These days splenectomies have become pretty routine surgical procedures and unless your spleen is very large or you are morbidly obese, the surgery is done via “keyhole surgery”. Laparoscopic surgery is done by making several very small incisions and this approach reduces the pain and recovery time significantly.
You can live a full life without a spleen, provided you take a few precautions. As we discussed above, the spleen is an important part of your infection fighting apparatus. Without a spleen you are less able to fight infections and it is important to remember that. At the first hint of a fever it is recommended that patients get themselves to the local emergency room. It is also important that some one goes with you to tell the ER doc in charge that you have had a Splenectomy. Otherwise the guy may roll his eyes and mutter about hypochondriacs who waste his time with a bit of a fever.
Things you can do to protect yourself
- If you have an enlarged spleen, it makes sense to avoid sports such as skiing, football and hockey. It is now even more important to wear a seat belt and be a safe driver.
- Avoid traveling to places that have endemic malaria infections. As I know from first hand experience, much of India has rampant malaria, especially during the rainy season when mosquitoes breed.
- If you have had a splenectomy, you might consider getting one of those medical bracelets that says so.
- Last but not least, please remember that while a splenectomy is not something a healthy person would want to do just for fun, in many CLL patients suffering from debilitating fatigue as a result of dropping red blood cell counts, getting rid of the diseased spleen will give you a new lease on life. Making these tough calls is what sets apart the survivors from the easy victims in this deadly serious CLL “game”.
- You can read a lot more about splenectomies at this site. http://en.wikipedia.org/wiki/Splenectomy
Off topic, my apologies for the long break between the last article and this one. I have been fighting a bit of a blue funk for the last little while. Hopefully it is now a thing of the past.