I am a 30-year-old female recently diagnosed with idiopathic thrombocytopenia purpura (ITP). I was experiencing some bleeding in my mouth and bruising easily. My hematologist prescribed Prednisone to raise my blood platelet count which is below 10,000, but I just found out the medicine is not working. It’s still below 10,000 after a week on the medicine.
He wants to do a bone marrow test at the end of the week and then possibly remove my spleen in a few weeks — if it is in fact ITP. Have you heard of them doing this before, and do you know what the success rate is? I am just so frightened of the bone marrow test, I have heard that it is very painful. Can other diseases cause your platelet count to be so low? Please help me understand this a little better.
Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disease affecting the blood platelets, also called the thrombocytes. When the blood platelet count falls too low, a type of bleeding into the skin called purpura often occurs. The medical name means: small pinpoint hemorrhages into the skin (purpura) caused by a very low platelet count (thrombocytopenic) of unknown cause (idiopathic).
An acute form of ITP occurs in children, often following a viral infection. It usually lasts only a few weeks, and may resolve spontaneously. Adults more often get a chronic form of the disease, occasionally associated with a viral infection such as hepatitis, Epstein-Barr viral infection, cytomegalovirus (CMV), HIV, or with the parasitic infection toxoplasmosis. In the majority of cases however, there is no such obvious initiating infection, and the cause is truly unknown.
Like most autoimmune diseases, women get ITP more often than men, in a ratio of about three to one. The most common age group affected is between 20 to 40, so you’re a pretty typical case. Often they have a history of easy bruising or very heavy periods, but both of these are very common symptoms and generally do not indicate thrombocytopenia.
People with the condition have an antibody in their blood that attaches to and incapacitates the platelets. Such platelets are routinely swept up and destroyed by the spleen, the organ that generally removes old or defective blood cells from the blood stream. When the platelet count falls into the range of 10 to 20,000 per ml, purpura may start to appear (our normal platelet count is between 150,000 to 400,000 per ml). Platelets are crucial to the complex system that causes our blood to clot, and they are constantly sticking to and plugging the minute holes we develop in our smallest blood vessels. It is the failure to close those tiny holes that allows the blood to ooze out of a capillary to form the tiny hemorrhages called petechiae that constitute purpura.
Despite the low platelet count and purpura, most people with ITP never develop life threatening bleeding. The exception is bleeding into the brain, which infrequently causes death. A mortality of one to three percent is usually reported for ITP.
Prednisone, a synthetic form of cortisone, is the standard treatment for ITP. About 50 percent of people with ITP will regain a normal platelet count on a high dose of prednisone, but when the prednisone dose is slowly dropped, most will experience a drop in the platelet count as well. Prednisone may need to be given for two to three weeks before a rise in the count is seen. People who don’t respond to prednisone like you, and those whose count falls when the prednisone is stopped are then candidates for splenectomy.
Your doctor wants to do the bone marrow exam to be sure that there is not some other condition causing the low platelet count. In the bone marrow, increased production of platelets should be seen, to compensate for their increased destruction in the blood. A failure to find overproduction or platlets, or problems with any of the other cells produced in the bone marrow would change the diagnosis, and splenectomy might no longer be the next treatment. Therefore, it is routine to do the bone marrow before proceeding with splenectomy or any of the other more serious treatments. If splenectomy does not bring about a rise in the platelet count, it may be necessary to search for an accessory spleen. If there is no accessory spleen, treatment with chemotherapy regimens may be necessary.
Rather than chemotherapy, many experts will advise no treatment at all if the platelet count stabilizes at 30,000 per ml or higher, since the risk of serious bleeding at that level is small. Aspirin and non-steroidal anti-inflammatory drugs such as ibuprofen or naproxyn should be avoided since they tend to interfere with the function of the few platelets remaining in the blood.