Or Tales of the Mega Spleen…
One of the sure signs that one might be suffering from Myelofibrosis is an increase in the size of the spleen. Yes, other things can cause the spleen to become enlarged. But when you have been monitored over the years for a blood disorder called Essential Thrombocythemia and your spleen starts getting larger, then chances are good that the development of MF is causing the spleen to expand.
Your spleen normally can be found nestled in your upper abdomen, under your rib cage. The so-called normal spleen is about the size of your fist. It quietly filters your blood, removing worn out red blood cells. It also acts as a reservoir for white blood cells and platelets. This allows quick dispersal of white cells in the event of an infection, or in the case of platelets, a quick release to help bring about prompt clotting to prevent excessive bleeding.
As MF progresses, the normal production of blood cells in the bone marrow is compromised. And then one of the amazing things about our bodies happens, the spleen attempts to take on the job of producing blood cells. Alas, MF has corrupted the “programming” and the cell production is not as good in quality or volume. The number of miss-shaped cells is increased and the ability of these red cells to carry out their main functions decreases. In a non-MF case, the spleen can do a fairly decent job at producing quality red cells.
The spleen is now encountering increasing numbers of misshapes and so it reverts to the primary function; that is to filter these out. And it gets a bit aggressive and starts filtering out more and more good red blood cells. And so it gets larger.
So, not only are your blood cell counts being thrown out of register, you constantly feel like you have just had three servings of the full turkey dinner. Your stomach is severely distended. Your red cell count has dropped, you are beyond tired and your heart rate is slightly elevated. Every movement is draining as you work to get oxygen through your system. Not much fun.
As the spleen increases in size, it starts to impact neighbouring organs. The liver starts to feel pressure from the spleen as the blood pressure increases with the increased volumes the spleen is forcing into the liver. The kidneys start to feel the pressure of the expanding spleen; your diaphragm and lungs start feeling pressure and one’s resting heart rate starts to increase. I have noted that mine has increased about twenty beats per minute. And then the stomach starts to be cramped. One feels full all the time and it becomes an effort to try and eat. Imagine going to a wonderful fish restaurant where the meals are a wonder to behold and enjoy and you can barely face a few slices of melon. I will occasionally force myself to have something amazing, like a hamburger without half the bun. Each mouthful is savored, knowing that for the next twelve hours the distended pain in my stomach will keep me awake all night. Worth it, oh yes indeed.
One can live with a slightly enlarged spleen; mine was enlarged by 4cm back in 2012. Did not bother me. But this started to change in early 2014 when I could feel my spleen getting larger. As it got larger, it impacts your abdomen, pressing against everything. This side effect of MF is referred to splenomegaly, or as I call it, the mega spleen.
I now look like I am three—four months pregnant and am in constant discomfort. But there is some relieve on the near horizon. There is a new drug that has shown an amazing ability to reduce the size of the spleen in MF cases. It is not a cure for the underlying cause, but it simply has shown to be very good a helping reduce the spleen to more manageable sizes with long-term use.
And tonight (Jan 15, 2015) I took my first 20Mg of Jakavi, but first I had to drop it onto the floor!
Myelo and Me 
How are you now? My mom is suffering from this.
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Greetings
Sorry to hear about your Mom, splenomegaly is not much fun. And it has far too many causes — do you know the source for your Mom?
When I wrote the column (Jan 2015) on Splenomegaly, I had been diagnosed Myelofibrosis. My hematologist thought Jakavi might help reduce the size of the spleen, but that treatment was interrupted by the discovery I also had Non-Hodgkin Lymphoma, which was also contributing to the size of my spleen. Six rounds of chemotherapy put the Lymphoma into remission. Today it is two years later, I’m still doing okay. Lymphoma still in remission and the Myelofibrosis is still slowly progressing. My spleen is still very large, and my oncologists have decided to reintroduce Jakavi in an effort to shrink the spleen. I really should write a more thorough piece on this.
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Hi Thank you for writing back so quickly.
My mom has polycythemia Vera. It was managed for the first few years with a monthly phlebotomy and then she started on hydroxy urea about 5 years ago. It was pretty well managed with that until last spring. Her spleen was growing but not interfering with life.
Then in June she had an emergency appendectomy and something bled internally and it took them days of transfusions and weeks of more terrible surgeries to finally get it to stop. They ceased the hydroxy to allow her huge abdominal wounds from the surgeries to heal, and simply treated her polycythemia with low dose aspirin. Since then, she has gone into septic shock twice, which can also enlarge the spleen.
Now that she is recovering from those ordeals, she has a megaspleen. Her WBC hovers around 70-90. She gets miserably distended after even a tiny meal. And you can see and feel her spleen bulging there next to her gigantic scars that have just healed.
Her hematologist wants to start her on Ruxolitinib. But she admitted that her other patient nearly died from the FDA recommended dose.
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Wow, your Mom is going through quite the ordeal. What can I tell you about Ruxolitinib, not much actually. I was put on it at 20mg twice daily back in January 2015, three weeks later it was reduced to 15mg twice daily. One week later, it was determined that I not only had myelofibrosis, but I also had non-hodgkin lymphoma (diffuse large B cell) and the two combined had greatly accelerated the increase in size of my spleen. The Ruxolitinib was discontinued really before it could be determined if it was reducing the size of the spleen, though I felt it had. My blood levels had collapsed to the point where the oncologist was concerned if I could survive a minor cut and had to remain in short distance of a major hospital. That was exciting to hear! Now, was it the Ruxolitinib or was it the lymphoma and leukaemia? Long story short, chemo and many transfusions later and I’m still here. My spleen has been slowly increasing in size to the point that the oncologists want to reintroduce the Ruxolitinib, I find out today at what dosage etc. They feel that my blood counts have recovered enough to withstand the 20 point drop that Ruxolitinib can cause. It is not known for sure if it will bring about a significant reduction in spleen volume, but it is all they have to offer at this point. My liver has also been increasing in size, the portal vein from the spleen to the liver has slightly increased in size due to increased pressure from the spleen, plus my liver now is forming blockages as a result of the myelofibrosis.
So for your Mom, not much fun with all this. If she begins the Ruxolitinib they will be closely monitoring her, possibly weekly blood tests at first. My oncologist says the results with other patients are favourable so, fingers crossed…
All the best wishes
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