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Have ET: What if spleen is enlarged?
Blood Cancers & Disorders | Last Active: Aug 21, 2022 | Replies (19)Comment receiving replies
@arti4 Arti4, my spleen has been enlarged for decades. That has caused me to take on many more infections over the years than should have been. After a time, I learned that this is a protein problem of light and heavy chains, and has affected every cell in my body. That problem turns out to be one of several forms of myeloma, such as amyloidosis, etc. It is a Prion disease, and is discoverable by a good medical team using a set of tests, including NTproBNP, Bindings sFLC, a PET scan and/or MRI of the heart. Several things will help you. Contact IMF.org, (international myeloma foundation) for their literature, especially their little handbook. Second, get at least, a genetic assay from a reputable (read Nebula Genomics or other Medicare-approved lab) that will include most rare diseases. I have had to even make threats of legal action against my doctor if he would not order the full genome test. Also, read and soak up the stuff from Mayo (Martha Grogan, especially), Karamanos, NIH, MD Andersen, etc, about Myeloma, especially amyloidosis. If your spleen ruptures, it will have to be removed, but you can live without it. But it is also affecting everything else: White Brain Matter, Heart, throat, lungs, liver, etc. So get on it, and quickly. old karl
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The International Myeloma Foundation is at myeloma.org. Your link took me to the International Monetary Fund. Slightly different deal. 🙂
Most ET patients do not have spleen enlargement, nor do they have myeloma.
The important genetic test for ET is for the JAK2 mutation. That's the most common mutation for ET. Patients who are JAK2+ are more likely to clot and my also progress to PV. Aspirin and HU may be given at lower platelet levels.
If you are JAK2 neg, like me, you should be tested for CALR and MPL mutations. CALR+ patients like me will not progress to PV and are less likely to clot. Consequently, CALR+ patients may not have to start HU until platelets rise to near 1,000. However, they will be prescribed aspirin or a substitute.
As with all my myeloproliferative neoplasms (ET, PV, and MF are the main ones), all roads could eventually lead to acute myeloid leukemia. At this time, I think interferon is the only treatment that actually seems to slow disease progression. Many older people do not tolerate interferon very well, but better dosing seems to be helping. Among older patients I know, moderate to severe depression seems to be the main side effect.
Stem cell transplant (SCT) is the only thing that can turn around acute myeloid leukemia, but it is a grueling ordeal, and success rates vary. My cousin, another MPN patient, age 60, was leukemia free but died from cardiac complications and pneumonia after SCT.