Hairy cell leukemia: What are the treatment options?

Hello, I was diagnosed in Dec 2013, I've only had chemo. The first round went well and with little discomfort other than the loss of some taste and smell. Ist round was just cladribine.
2nd round some 5-6 years later, I was given some rituximab but had a bad reaction to it. No one has been specific about the reaction and how it affected nerves in my shoulders. I finished my week with just the cladribine. The use of my shoulders came back and I'm back to playing hockey and golf.
HCL can be managed.

@legacyrock, I add my welcome along with @ptannie. I'm also tagging additional members with experience with hairy cell leukemia like @c1608 @ptannie @jacg and @tallyteresa.

As you may have already been told, treatment for hairy cell leukemia doesn't always need to start right away if you're not experiencing any symptoms.

Legacyrock, how was your diagnosis discovered? Are you experiencing symptoms? Is treatment being suggested for you at this time?

@, legacyrock, there are studies being done using non chemo type drugs. I was diagnosed in November 2022 and needed immediate treatment due to critically low blood cell counts. I received 5 consecutive days of a chemo infusion of cladribine and then 8 weekly infusions of a monoclonal antibody, rituximab, which has a long track record of use for arthritis and other cancers. I achieved remission as evidenced by recovered blood counts and a followup bone marrow biopsy. Hairy cell leukemia (HCL) has a very high success rate for treatment and I can expect to go at least 10 years before requiring re-treatment. It is a chronic condition and monitoring of cell blood counts is essential to ongoing management. Dr. Kreitman at NIH is the leading expert and researcher for HCL. He happens to be doing a session for the HCL foundation on Nov. 8 and I strongly suggest that to you,
https://www.hairycellleukemia.org/calendar He will likely speak to the other non-chemo options being studied. He can also review your case and offer suggestions. I only found him after starting my chemo and have joined a study on HCL and COVID as well as benefitting from his insights on care/response along the way. I have found great support from a Facebook group too - hearing insights that my doc couldn't know for such a rare condition. I can also highly recommend it as well, https://www.facebook.com/groups/hairycellleukemiasupport. You are not alone - lean on those of use that have gone before, whether months or years beyond the scary parts.

The discovery of the HCL was a strange series of events. The first year of covid I had to have emergency surgery for a part of my small intestine that the blood supply was being cut off. Months later my oldest daughter who is a paramedic read through the reports pertaining to the surgery. She asked me what they were doing about the aneurysm? I didn’t know anything about it. Sent a message to my primary doc who scheduled another ct scan for the aneurysm . At that time we noticed some lymph nodes that were larger than they should be. Had labs done at the same time and numbers were low but not concerning enough to do anything but keep a eye on them. 3 months ago at another ct scan discovered very swollen lymph node in my armpit. Labs indicated numbers were a bit lower. Biopsy of the lymph node is when I received the diagnosis of HCL. Kept checking numbers and ultimately had bone marrow biopsy on Monday which confirmed everything. I have had no symptoms. Appetite is good, energy is good, sleep is good. Basically I feel fine and if it hadn’t been for my daughter reading my post surgery report I wouldn’t have any idea that anything was wrong.

I did join the fb group a few days ago. After researching Hcl and reading med papers I was fairly certain that chemo is not the only option. I want to avoid chemo if at all possible. During the appointment with oncologist I asked about alternative treatments. He obviously didn’t like that question. After talking to him a nurse came in and tried to turn the fear meter to 11. She also took it upon herself to schedule appointments starting in 3 days for chemo and the following weekly treatments. I was/am po’d that they wouldn’t give a person time to digest everything before making a decision about treatment.

Yeah, that should be a red flag when an oncologist bristles at questions about treatment options. I'm not saying there is a conflict of interest, but they do bring in a huge amount of money to a cancer center by administering chemo. I do have peripheral neuropathy as a result of the chemo, which rarely happens with only 5 days of it. Timing of treatment really should be based on how you feel along with the CBC numbers (hemoglobin < 11g/dL, platelets < 100,000/mcL, absolute neutrophil count < 1000/mcL). Dr. Kreitman is very responsive to inquiries and might be able to help in your decision, kreitmar@mail.nih.gov. With your research, you may be aware that it will be very helpful for him to know if you have HCL (BRAF +) or HCL variant (BRAF -) which you would learn from your bone marrow biopsy.

Thank You. I’ll see if I can determine which variant it is, if not I assume I can provide him a copy of the results. A friend of my daughter was diagnosed with Hcl a little over two weeks ago and is still in the hospital. He had gone to urgent care because he just didn’t feel right. They sent him to the ER to get labs done and his numbers were so low they immediately started a blood transfusion. He had a total of three transfusions and then was started on the chemo regimen. I haven’t heard why he’s still in the hospital.

Do you know what I need to look for to determine the variant?

I didn't get the full biopsy report until 11 days after the procedure. Initial report showed BRAF negative, but the final was positive. See this from a slide from a HCL.org presentation:

Thank You,
The only matching number I have from those is CD20. I will try and get ahold of Dr. Kreitman.