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Systemic Mastocytosis Is a Rare but Treatable Condition

Dear Dr. Roach: I am a 66-year-old white male. About 15 years ago, I was diagnosed with adult-onset indolent systemic mastocytosis. I probably contracted it at least 10 years before that. Few doctors even know what mastocytosis is. I was told at the time that few people have it and that there is no known cause and no known cure. Since then, has research shown any possibility of a cure on the horizon? — J.K.

Answer: Mastocystosis is a rare disorder of mast cells. It is called cutaneous mastocytosis when it is limited to the skin, and systemic mastocytosis when it affects organs, with or without the skin. Skin symptoms usually are itching and rash, especially after heat or trauma.

Mast cells are part of the body’s inflammatory system, and they can release many substances to fight off invaders, like bacteria; however, inappropriate release of these chemicals, such as histamine, causes allergic reactions that can be severe. Most people with mastocytosis have a mutation in a gene called c-kit, but how that causes the symptoms of the disease still is incompletely understood.

Systemic mastocytosis most commonly affects the bone marrow, liver or spleen. Symptoms of systemic mastocytosis may look like severe allergic responses, from release of the substances of the mast cells. The symptoms of flushing and itching can be associated with more worrisome symptoms of fainting, abdominal pain, nausea, diarrhea or vomiting. Depression and mood changes also are common in this condition, and can be ignored by doctors who don’t understand the condition.

You are correct that there is no cure, but there are treatments that can improve your quality of life. Probably the most important is to recognize and avoid the triggers of mast-cell release. Medications to counter the mast cell contents, such as antihistamines and cromolyn, should be taken at onset of symptoms. Up to one-half of all adults with mastocytosis can have anaphylaxis, the most dangerous of all allergic-type reactions, which can present with shock and severe swelling. If the swelling affects the throat and airway, it can be fatal. Thus, all people with mastocytosis should have epinephrine (at least two doses) available at all times. A medical bracelet also is a smart idea.

I don’t know of any cures on the horizon. Some newer drugs are becoming available. More information is at www.tmsforacure.org.

Dear Dr. Roach: In our paper, you had an article from a man that said he had his prostate removed and was wondering about taking testosterone. You said he would likely have an increased risk of prostate cancer recurrence.

I, too, have had my prostate removed and was wondering how you could have an increased risk of prostate cancer after having the prostate removed. Could you explain, please? — C.G.

Answer: That is a very fair question. The answer is that even the best surgery can leave some prostate tissue in place. It’s also possible that some tumor cells spread before surgery. Any residual cancer can be stimulated, in theory, by taking testosterone. Fortunately, the available evidence so far suggests that this risk may be much lower than feared. Careful follow-up remains essential.

Dear Dr. Roach: Recently I was told that the “new way” to take blood pressure readings was to hold your arm against your chest and over your heart. I have not seen this on the web anywhere. Is this true? — W.S.

Answer: It’s not so new: We have long known that the arm should be at the same height as the heart. The arm also should be supported. Otherwise, the readings will be slightly (or not so slightly) off.

Dr. Roach regrets that he is unable to answer individual letters, but will incorporate them in the column whenever possible. Readers may email questions to ToYourGoodHealth@med.cornell.edu or write to P.O. Box 536475, Orlando, FL 32853-6475.