Adult Langerhans cell Histiocytosis and CMML

Posted by cblowers1 @cblowers1, Feb 27, 2020

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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I too have been diagnosed with Langerhans Cell Histiocytosis. I would like to contact Dr Go regarding further testing and treatment. My local oncologist does not know what my treatment plan should be. I was diagnosed after having two lymph nodes removed.

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Hello @painterderek and welcome to Mayo Connect.

I am sorry to hear that your current doctor does not know what your treatment plan should be. I am glad, however, that you are looking for a second opinion from a doctor at Mayo who is experienced with this disorder. Here is a link to the appointment desk at Mayo http://mayocl.in/1mtmR63.

For your information, here is a link to Mayo's website that will give you more information about Dr. Go., https://www.mayo.edu/research/faculty/go-ronald-s-m-d/bio-20013527

If you are comfortable doing so, would you post a bit about your history with Langerhans Cell Histiocytosis? I'm thinking of things like your original symptoms, the tests that led to this diagnosis, any treatments that your current doctor has suggested, and how you are feeling now.

I look forward to hearing from you again.

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My symptoms started 3 years ago with severe dandruff on the sides of my head. Later I had
large red dry red spots on my stomach, lower back,and legs. I went to a dermatologist who
said I had plaque psoriasis. On Sept of 2020 I noticed one of my lymph nodes was bard.
A surgeon removed two that were suspected and I U Med Ctr diagnosed me with LCH.
My oncologist is talking about chemotherapy or prednisone for my treatment. He has confirmed
With Mayo 2.5 weeks ago and still waiting for second opinion.

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@painterderek, I'd like to add my welcome to Mayo Clinic Connect. I'm tagging @cblowers1 to ensure that they see your message and they can share their experience at Mayo with Dr. Go. I'm glad that your oncologist is consulting treatment options with Mayo Clinic.

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My Fiance was recently diagnosed with Langerhans cell Histiocytosis. We are in search of a second opinion to be sure of the diagnosis and treatment. The doctor is wanting to treat with Chemotherapy (low dose infusion, 5 straight days a week once a month for 12 months) I get the feeling that since this is a rare disorder/cancer they don't seem to know a lot about it and I want to make sure that this is the necessary treatment. This all started a year and a half ago with an itch. With that being said he has also been diagnosed with Alpha Gal from a tick bite. Apparently he had a much going on all at the same time and his bloodwork is kinda all over the place. Does anyone have any further information or experience with LCH? I will take all the input we can get.

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@christina67

My Fiance was recently diagnosed with Langerhans cell Histiocytosis. We are in search of a second opinion to be sure of the diagnosis and treatment. The doctor is wanting to treat with Chemotherapy (low dose infusion, 5 straight days a week once a month for 12 months) I get the feeling that since this is a rare disorder/cancer they don't seem to know a lot about it and I want to make sure that this is the necessary treatment. This all started a year and a half ago with an itch. With that being said he has also been diagnosed with Alpha Gal from a tick bite. Apparently he had a much going on all at the same time and his bloodwork is kinda all over the place. Does anyone have any further information or experience with LCH? I will take all the input we can get.

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Hi Christina, welcome. You are wise to seek a second opinion from an institution with experience in rare cancers, like Mayo Clinic or a cancer center of excellence. To get a second opinion from a doctor at Mayo Clinic who is experienced with this disorder, here is a link to more information about requesting an appointment http://mayocl.in/1mtmR63.

How is your boyfriend feeling?

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I was diagnosed with LCH at Mayo Clinic in 2016. I have skin only. After several topical treatments which caused the lesions to become more painful and ulcerated, I began taking hydroxyurea. I have taken it for 2 years with no real side effects and the skin lesions are very faint and no itch or pain-a miracle for me.
I recommend going to histio.org , the website of the Histiocytosis Association for reliable information. You can find a link there to a recently released NCCN protocol for treatment of adult histiocytosis diseases. (There are three other diseases that are even rarer). There are also doctors listed that have special interest in histiocytosis.
There are also closed Facebook groups such as Adult Histio Warriors where people share their experiences with the disease.
It is difficult because it is rare and it manifests itself in so many different ways. It is so rare that diagnosis usually is years after first signs. Funds for research are scarce since so few people would benefit. Good luck!

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@colleenyoung

Hi Christina, welcome. You are wise to seek a second opinion from an institution with experience in rare cancers, like Mayo Clinic or a cancer center of excellence. To get a second opinion from a doctor at Mayo Clinic who is experienced with this disorder, here is a link to more information about requesting an appointment http://mayocl.in/1mtmR63.

How is your boyfriend feeling?

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Actually he is feeling very good. This all started with an severe all over itch. We have seen a dermatologist, hematologist, a urologist, an allergist. He has had numerous blood draws, a bone marrow biopsy, a lymph node biopsy, a skin biopsy etc. Along the way he was also diagnosed with Alpha Gal from a tick bite. He has since avoided red meat and pork and the itch has gone away. Since the itch was his biggest issue he is back to his old self and feeling great. Which furthermore confuses us on the diagnosis and treatment. I have read that Langerhans has still not been determined if it is a cancer or a blood disorder, but yet they want to treat it with 12 months of Chemo therapy. That seems like a bit much to me. If they aren't sure if it is a cancer, then why Chemo? Are we killing cancer or suppressing the immune system? If we are suppressing the immune system, are there not other things that do that besides chemo? I have also read that this is more common in children, however many children do not need treatment, that it goes away on its own or they outgrow it. So if that is the case in children then what changes when you are an adult? We just want to make sure that he actually has LCH, and that this is the correct treatment for it. Thank you for your reply and I am sure we will be reaching out to Mayo Clinic in the near future for a second opinion. What do we need to do to get started? Can we send all of the test results over to be reviewed before we make an appointment or does the appointment need to come first? Thank You

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@christina67

Actually he is feeling very good. This all started with an severe all over itch. We have seen a dermatologist, hematologist, a urologist, an allergist. He has had numerous blood draws, a bone marrow biopsy, a lymph node biopsy, a skin biopsy etc. Along the way he was also diagnosed with Alpha Gal from a tick bite. He has since avoided red meat and pork and the itch has gone away. Since the itch was his biggest issue he is back to his old self and feeling great. Which furthermore confuses us on the diagnosis and treatment. I have read that Langerhans has still not been determined if it is a cancer or a blood disorder, but yet they want to treat it with 12 months of Chemo therapy. That seems like a bit much to me. If they aren't sure if it is a cancer, then why Chemo? Are we killing cancer or suppressing the immune system? If we are suppressing the immune system, are there not other things that do that besides chemo? I have also read that this is more common in children, however many children do not need treatment, that it goes away on its own or they outgrow it. So if that is the case in children then what changes when you are an adult? We just want to make sure that he actually has LCH, and that this is the correct treatment for it. Thank you for your reply and I am sure we will be reaching out to Mayo Clinic in the near future for a second opinion. What do we need to do to get started? Can we send all of the test results over to be reviewed before we make an appointment or does the appointment need to come first? Thank You

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Hi Christina, apologies for the delay in responding. You’re asking all the right questions and I hope you’ve received answers from experts in the meantime. Chemo drugs are mostly associated with cancer treatment, but can be helpful with other conditions, including auto-immune conditions. @becsbuddy and @loribmt may be able to tell you more about this.

Have you already contacted Mayo Clinic for a second option? They will walk you through the procedure of sending test results and medical records before your initial consult.

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I have spoken with them, but they are waiting for him to call and schedule something. He has his own business so it is not easy for him to get away for a week and we are quite a distance away. I am afraid that he feels so good that he is dragging his feet and a bit in denial of his possible diagnosis. Hoping to get something scheduled soon.

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