August 2010

In your opinion what is the best treatment approach in the following case:
A 32 year old male patient diagnosed with hairy cell leukemia, received Cladribine as first line treatment. He achieved complete remission, relapsed after 8 months, received another course of Cladribine, achieved complete remission, and then relapsed after 2 years.

In general, the treatment of hairy cell leukemia at relapse depends upon the quality and the duration of the response to the previous treatment. It would be important to know whether or not the patient achieved a complete remission following the initial course of Cladribine. It would be important to know more about the characteristics of the leukemia at diagnosis.

Furthermore, more information regarding the bone marrow findings at the time of remission and relapse would be helpful. The second remission lasted somewhat longer, but still is in the range to consider alternative treatments. There have been a number of reports discussing the treatment of hairy cell leukemia at relapse, and how to approach patients following short remissions. Considering the young age of the patient, the potential for discussing the details of this case with one of the centers listed in our Centers of Excellence section provides another opportunity for your hematologist to discuss the best approach to this situation.

My husband, age 72, was diagnosed about 6 months ago with HCL. He is not being treated yet. His blood counts are monitored monthly. Can I expect him to live a normal life. Right now all he has is some tiredness in the early evening and bruising.

The treatment of hairy cell leukemia has made enormous progress over the past 25 years. Patients who need to be treated have a high chance of responding with durable remissions. In patients who do respond, the quality and quantity of life may be close to what would have been their normal life expectancy.

There are many considerations that go into the decision to treat hairy cell leukemia. Some patients may be closely followed if their blood counts are not low enough to merit starting therapy. If patients are not having symptoms and have near normal blood counts, the hematologist may elect to follow the patient closely. If blood counts are declining, then the decision to treat will be based upon the risks of treatment compared to no treatment. It would be important to let his hematologist know about the bruising.

Some patients have other medical conditions that could complicate treatment. So, the decision to treat needs to take into consideration such important medical conditions as kidney function and whether or not there is any evidence of infection. It would be best to ask your hematologist to help answer these questions because he or she will have all of the facts of your husband’s medical condition.

I was diagnosed with a blood clot in 2001. I am on medication, currently 12.5mg of Warfarin, which seems high to me. In 2010 I was diagnosed with hairy cell leukemia. Any relationship between the clot and hairy cell? Aren't both clotting, Vit K issues? Am I unique? Is Warfarin prescribed to someone with hairy cell? Or is there just no connection? By the way diagnosis was 6/1/2010 and blood counts have not yet fallen to levels where treatment would begin, according to my doctor.

Several patients have been reported in the literature to have a disorder of blood coagulation in addition to hairy cell leukemia. While these cases have been reported, it is not clear how often this occurs. It would be best to ask your hematologist whether or not there is any relationship of the earlier blood clot and the hairy cell leukemia. From the information provided, it is difficult for us to answer this question. If a patient is on long-term Warfarin, the hematologist may have made a diagnosis of a condition that predisposes the patient to subsequent blood clots. Some of the disorders associated with increased risk of blood clotting can be seen in patients with underlying malignancy. Therefore, your hematologist would be in the best position to comment if there is any possible relationship in your case.

With respect to treating patients with Warfarin for a condition that predisposes to blood clotting, it is important to make certain that you are following your platelet counts as well as your coagulation studies carefully under your physician’s supervision. Patients with hairy cell leukemia who do not have low blood counts can be followed without treatment for the leukemia. However, it is important to have surveillance of the blood counts, in particular the platelet count. Because the leukemia and its treatment may lower the blood counts, a careful decision of when to treat will be based upon your actual laboratory values. Your physician will make the decision on when to treat the hairy cell leukemia.

My brother-in-law was recently diagnosed with HCL and started chemo last week. He is expecting to go home sometime this week. His home is really a cabin in the woods, latrine, generator for intermittent power, no running water.

Is he putting himself at higher risk of infection, reaction, whatever, by being in such an environment? Do you think an HCL patient can manage in such a place when there is so much work to be done for everyday subsistence? This lifestyle choice made sense before his diagnosis, but I'm concerned for him now.

It is understandable that you are concerned about your brother-in-law. Following treatment for hairy cell leukemia, the blood counts may go lower before they are better. Fortunately, the response rate is high. Patients usually obtain a response, but the blood counts can be reduced for weeks. It is difficult to know how much his blood counts will go down because we do not have sufficient information to give a specific response.

While the blood counts are low, there is a risk of serious infection or bleeding. Under these circumstances, it would be better if the patient could stay with a friend or family member to ensure that help could promptly be obtained if needed. If blood counts are reduced, frequent blood tests are usually obtained. Patients check their body temperature several times a day to avoid missing an early infection.

In a remote location, it may be difficult to have cell phone reception. If the patient becomes ill or develops a fever, it may be difficult to get help in a reasonable period of time. Therefore, temporary arrangements for living with someone may help make his recovery safer.

Living in a remote area does increase the risk of lowered blood counts. The primary concern to me would be how to arrange for help if needed. Without knowing more of the details of his medical condition or his actual environmental health risks, it is hard to be more concrete in providing recommendations. Because the results of treatment are very effective with this disease, it would be reasonable to ask him to make some plans for living close to help if needed until recovery is solidified.

I am a 62 year old male, diagnosed with HCL and treated with Cladribine in February of 2002. My blood counts have been on a roller coaster, with small variances, since treatment but have stayed free of HCL. In 2005 the doctors found several nodules in my Thyroid and tracked them with yearly sonograms.

In December 2009, I was referred to the Thyroid Surgeon who removed the right half and 2/5 of the left side. Test results indicated cancer and they decided to remove the rest of the left and do a dissection of the interior of left neck to biopsy some nodules also found on a follow up CAT scan. This last operation affected my vocal cords leaving me with just a horse whisper, hey but it could have been worse.

My blood counts are “low but stable” according to my oncologist. The thyroid counts are really high and the endocrinologist is working on adjusting medication to bring them in limits. These two doctors are proposing doing iodine radiation as soon as the counts get better. They did the surgery to remove all tissue as possible to be able to use a lower dose during the treatment. They are telling me that there is not much previous treatment so they are not sure what this will do to my HCL.

I understand that the radiation will kill all my white cells which cause me much concern because of infection. As it stands I have been allergic to every anti-biotic that they have used on me the last 10 years. I am currently looking for an Allergist my area since the VA does not have one on staff. Can you give me any direction or information on previous patients in a similar position? The Doctors tell me that I am a really rare patient. By the way the Veterans Affairs gave me an additional 100% disability due to my exposure to Agent Orange in Viet Nam. Thank you for your information.

It is encouraging that you indicate that your hairy cell leukemia responded to the Cladribine. You indicated that your blood counts have been on a “roller coaster” and that they have been “low.” This raises the concern that your bone marrow may be sensitive to irradiation. Furthermore, you said that your doctors are concerned about your low blood counts and that the irradiation may lower them to a dangerous level.

Your doctors have said that your case is rare. In general, hairy cell leukemia is a rare form of adult leukemia. There are reports in the literature that patients with hairy cell leukemia may be at increased risk for developing thyroid cancer. Despite this increased risk, there are not many reported patients who have had the challenge of using irradiation for thyroid cancer after being in remission with Cladribine.

In an effort to adequately address your question, a thyroid cancer specialist was contacted. Without knowing all of the details of your case, it is not possible to make very specific recommendations. For example, we do not know the exact results of your blood counts. The dose of I-131 irradiation ultimately delivered may depend in part on risks for the thyroid cancer. In addition to knowing the tumor type, it would be important to consider the patient’s age, stage, serum thyroglobulin, and scan results. Considering the concerns with your blood counts, the dose selected is important. If there is compromised bone marrow reserve, the use of dosimetry could be incorporated into the plan to improve the safety of the treatment. There are centers with extensive experience in treating thyroid cancer throughout the nation, and they could be consulted for advice.

I was diagnosed with HCL in 1988 and had my 5th relapse last autumn. The three latest relapses came once every 12 months. I have been treated with Cladribine and Rituximab. Can HCL become acute and more dangerous? What can I be treated with if I continue to have one relapse per year?

While the response rate is initially good after relapsing, responses may eventually become shorter after being treated with the same medication. In addition, the response rate may fall after repeated relapses using the same medication. Cladribine (2 CDA) is a highly effective medication for this disease. However, repeated courses may eventually cause some problems with bone marrow recovery. You have received Rituximab, and it is important to understand your response to this drug. Finally, there is another drug (Pentostatin) that has been used after patients have relapsed from Cladribine. Your doctor would need to help answer the question of what treatment options there are for you.

There are many clinical aspects of your case that are not covered in your note. It would be important to know how your blood counts are doing. Re-treatment for hairy cell leukemia in relapse often depends upon the actual blood counts. If the blood counts are falling, then re-treatment may be recommended depending upon the actual counts. It is also important to know what the latest bone marrow shows. Sometimes, there are other problems in addition to hairy cell leukemia that can contribute to lowering of the blood counts. Finally, it would be important to know if your spleen is enlarged. Therefore, it would be important to discuss the details of your case with your doctor. If your doctor is interested in getting advice, there is a list of the Centers of Excellence on Hairy Cell Leukemia here on the website. This list provides contact information for doctors who may be able to provide assistance.

I was diagnosed with HCL in 2005 at age 49, and had 30% HCL cells in my Bone marrow. I was administered Cladribine for 7 days, and recovered well from the treatment with my platelets and white blood count returning to normal. My levels since treatment have gone down and I received another treatment of Cladribine in Feb 2010. I had 15% HCL this time in my Bone Marrow. I have not responded at all to this treatment, and my blood levels continue to be low.

I'm scheduled for another bone marrow biopsy on 1 Sept 2010. Don't know what will come of this but I do have several questions. If I still show 15% HCL, what would be the best course of treatment in my case?

I would also like to know if there's an Oncologist that specializes in HCL that may be in my best interest to visit for a cure. I have complete confidence in my oncologist, but finding one that is more up to date with HCL could be beneficial in the long run. Just want to get your opinion so I know all the options available to me.

It is unusual not to respond to a second cycle of Cladribine. Approximately 90% of patients who relapse after a first cycle of Cladribine respond well to a second cycle (and about 60% respond with a complete remission, that is complete eradication of all of the hairy cells). After a second cycle of Cladribine, the blood counts may remain somewhat below absolutely normal levels after a cycle and it is important to determine whether a patient indeed does not respond to the second cycle of Cladribine or whether the patient responded, but the blood counts remains somewhat low. A repeat marrow should be carried out to make this determination.

If the blood counts are adequate it may be that a patient does not need further treatment even if there is a small amount of hairy cell in the bone marrow. If the patient has not responded to the second cycle of Cladribine, and the blood counts are abnormal enough that treatment is needed, there are a number of possible treatments. The new immunoconjugates, HA22 and BL22 are antibodies linked to potent chemicals which are toxic against the hairy cell. These are remarkably active in patients who have relapsed after several cycles of Cladribine. They are currently only available as a clinical trial being conducted at a very few selected centers since the agents are not approved by the FDA and are not commercially available yet.

If these new agents are not available or a patient is not eligible, one could consider the monoclonal antibody Rituximab. The ability of this agent to eradicate all or most of the hairy cells in the bone marrow is not as high as we all would like it to be, but may range from 25% to 80%. There are a number of physicians who specialize in the treatment of patients with hairy cell leukemia and you are more than welcome to contact any of us directly through our Centers of Excellence for further information. As always, you should consult with your current physician regarding your treatment.

Mailbags

HCL Forum

Ask an Expert