Adult Langerhans cell Histiocytosis and CMML

Posted by cblowers1 @cblowers1, Feb 27 3:54pm

I was diagnosed with Langerhans cell Histiocytosis in May of 2016 after a biopsy of skin lesions. I had never heard of this rare disease and the NP at Mayo Rochester who called with results said she had had to look it up and she gave me some basic information. I soon learned much about this blood disorder of myeloid cell origin which affects only 1 or 2 adults per million population. It is more commonly a pediatric disease, although still rare. It can cause lesions on skin, bones, lungs, liver, or spleen. It can also cause problems with the central nervous system.
After a PET scan and other tests and imaging, it appears that mine is skin only. I did test positive for an acquired mutation of the BRAF gene that is found in up to 60% of patients with the disease. I have tried numerous topical treatments, including several strong steroidal ointments and cream, ointment to rev up my immune system and even nitrogen mustard in gel form. Although some helped, all topical treatments eventually, or very quickly caused worse skin irritation that the original lesions. Brachytherapy to affected skin in the groin area caused painful burns. For the last 5 months, I have been taking Hydrea, 500mg and then 1000mg per day.
This has resulted in the best response yet. I still seem to have flare ups and then subsiding of the skins, but much less severe. In June, 2019, a bone marrow biopsy was performed after blood labs had shown low platelet counts and high monocytes over a period of time. I was diagnosed with chronic myelomonocytic leukemia (CMML 0). This is another rare disease that is a myeloid disorder. My best hope is for a slow progression of this leukemia, since current treatments only treat symptoms. There are possible treatments being studied, but not yet available. Having any rare disease is frustrating since there is often very little money available for research because so few would benefit. It is also difficult to find providers who are knowledgeable about diseases so rarely seen! Any others have any experience with these disorders?

Hello @cblowers1. I'd like to start by inviting @javajude, who recently talked about their son going in for treatment for CMML. I'd like to also invite back to Connect, @teammeadows, @jzier, and @tbone45 who did mention they had CMML as well.

I had an opportunity to sit down with Dr. Go of Mayo Clinic Rochester to discuss Erdheim-Chester disease and the histiocytosis working group here, https://connect.mayoclinic.org/page/hematology/newsfeed-post/erdheim-chester-disease-ecd-what-is-it-and-what-are-the-signs-and-symptoms/. Included in that blog is the Mayo Clinic Histiocytosis Working Group Consensus Statement for the Diagnosis and Evaluation of Adult Patients With Histiocytic Neoplasms: Erdheim-Chester Disease, Langerhans Cell Histiocytosis, and Rosai-Dorfman Disease, https://www.mayoclinicproceedings.org/article/S0025-6196(19)30273-3/fulltext that does contain a lot of information on the histiocytosis diseases and may be of some interest to you.

@cblowers1, you mentioned the hydrea has helped manage the symptoms outside of occasional flare ups. Has this allowed you to return to a more 'normal' routine and activity? How do you tolerate the drug?

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@JustinMcClanahan

Hello @cblowers1. I'd like to start by inviting @javajude, who recently talked about their son going in for treatment for CMML. I'd like to also invite back to Connect, @teammeadows, @jzier, and @tbone45 who did mention they had CMML as well.

I had an opportunity to sit down with Dr. Go of Mayo Clinic Rochester to discuss Erdheim-Chester disease and the histiocytosis working group here, https://connect.mayoclinic.org/page/hematology/newsfeed-post/erdheim-chester-disease-ecd-what-is-it-and-what-are-the-signs-and-symptoms/. Included in that blog is the Mayo Clinic Histiocytosis Working Group Consensus Statement for the Diagnosis and Evaluation of Adult Patients With Histiocytic Neoplasms: Erdheim-Chester Disease, Langerhans Cell Histiocytosis, and Rosai-Dorfman Disease, https://www.mayoclinicproceedings.org/article/S0025-6196(19)30273-3/fulltext that does contain a lot of information on the histiocytosis diseases and may be of some interest to you.

@cblowers1, you mentioned the hydrea has helped manage the symptoms outside of occasional flare ups. Has this allowed you to return to a more 'normal' routine and activity? How do you tolerate the drug?

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At my annual clinic visit last summer I met with Dr. Go at Mayo, Rochester, and we had a good discussion concerning possible treatments for the Langerhans cell Histiocytosis. My LCH was diagnosed at Mayo in 2016. My CMML had just been diagnosed. I am receiving most of my care at Dana Farber Cancer Institute in Boston because it is closer to my home, however, I still visit Mayo Clinic annually and Dr Go said that he planned to discuss my case with the histiocyte disorders task force.
I have been taking hydroxyurea capsules since mid September and have experienced minimal side effects. My doctor recommended taking it at bedtime, since nausea is often experienced and he thought that I might sleep through that. I have not noticed that problem. I wake up in the night with a headache about 2-3 times a week. These headaches are not severe and if they are not gone by the time I get up in the morning, they are gone by 11 or so in the morning. I had not had headaches in several years, so I blame it on the Hydrea, although it could be from the blood diseases.

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