Question: I am right now 2 years out on my last bone marrow biopsy that said no HCL found at all nor non-HL. My blood test of two weeks ago showed perfect except for low leucocytes. (589) Is this normal and will it rise as time goes on? Can I do anything to raise my count? My HCL was diagnosed in May 2013 and the clear bone marrow biopsy was in that Sept. Cladribine for 7 days was my treatment and 14 days Neupogen.

Answer: A normal leukocyte level number ranges from 3,500 to 10,500 in a microliter of blood or 3.5-10.5 x 10 9/L. The final number depends on your race, age, and time of day that it was tested. The statement: “except for low leucocytes (589)” is a bit difficult to understand depending on the measurement as we don’t routinely see the number expressed like that—does the level actually show 589 white blood cells in a microliter of blood? How is the differential count of the white blood cells? Are the neutrophils or the lymphocytes low? (neutrophils and lymphocytes are subtypes of white blood cells). In any event, is it expected that chemotherapy can bring your numbers low (your white cells, your red blood cells, and your platelet count can go low requiring transfusions or the use of growth factors like neupogen). It takes several weeks to months to completely allow your bone marrow to recover its fully functioning state. Since the last time you had chemotherapy was 2 years ago, it is possible that your doctor just wants to see the trend of the white blood cells over time. You didn’t mention if you had any new symptoms or an infection, but I am assuming no other symptoms were present at your last visit. We have had patients with HCL that had an episode of low white blood cells that over time resolved on its own—we only monitored their numbers and after a while the numbers went back to a normal level. If the number of white blood cells remains in the low level, your doctor may want to repeat a bone marrow biopsy to see if there is evidence of disease recurrence or another reason for the low white blood cells—sometimes certain medications can affect your total number of white blood cells.

Question: If a patient has had a complete response to treatment, will he/she now test negative for the BRAF mutation? Or once you have the mutation you will always test positive?

Answer: BRAF mutation is only present in leukemic cells. So if there is no residual disease no BRAF will be detected by regular analysis. Unless they use BRAF mutation as a target of quantitative /digital PCR and use it as a sensitive measure of residual disease (MRD) in a way similar to BCR-ABL quantitative monitoring in patients with CML or ALL.

Question: I’m 46 years old and found out two weeks ago that I have HCL. I begin treatment with Cladribine tomorrow, two hours per day for five consecutive days. I feel great; I’ve been completely asymptomatic. The CBC and bone marrow biopsy are the only signs that anything’s wrong with me. I know I need to be cautious of foods that could be a source of food-bourne illness. Are there any foods I should avoid during the treatment to prevent any interaction with the chemo, or any particular diet that will help achieve a better outcome? I typically spend 30 minutes on the treadmill 3-4 times per week doing moderately intense workouts. I quit going to the gym about three weeks ago because I’m a little germophobic, but I still take a couple of 20 minute walks per day. I know it’s good for me overall, but is there any research that indicates whether or not the effectiveness of my treatment will be affected by that for better or worse?

Answer: No, there are really no foods you need to avoid that would interact. Although rare, certain foods/cheese may react with some chemo, but I am aware of no such interaction with cladribine. No adverse effect to exercise. Keep it up.

Question: There is one question I have that you might be able to help me with. In November I completed my first course of Cladribine after being diagnosed with hairy cell. In February I had a SPEP test and it was all negative. I just repeated that test and it is now positive IgG kappa, only a very minor amount but it is concerning me that it might be an early sign my HCL is transforming. So I wanted to know if it is common to find MGUS’s in HCL patients because of the existing leukemia or if it is an unusual occurrence. I would be very grateful for any knowledge you could pass on to me on this matter.

Answer: The presence of a monoclonal component is not frequent but not unusual. Generally this finding is not associate to the progression of the disease. We can’t exclude the possibility that it is a transitori finding. In conclusion, I would be very tranquille and every 6 months check the lab test.

Question: I’m 54 and was treated with Cladribine in early 2012. Since treatment my white blood count has not returned to normal and hovers around 2.0.My platelets have historically run between 140 to 180, my last blood work in May they were 110; ABS NEU usually are between 1.0 to 1.7, most recent was .77; ABS Lymphocyte actually increased to a new recent high of .84; Hemoglobin was in usual range of 14.8; white count was at 1.6 which is low for me but have been that low a couple times before in last year. I’m due for my next blood work mid-July. My oncologist did not indicate any concern after my most recent test, but a downturn with three of the key markers has me a little more concerned than normal. I know I will have to re-treat at some point in the future since I’ve only done one round of 7 day treatment with Cladribine, but I’m hoping I can get a few more years before repeating. Any feedback provided would be greatly appreciated.

Answer: It’s sometimes difficult to determine the significance of CBC at one time point. However, as you indicated, a trend means much more. So I would wait for your next blood count in July before jumping into any conclusion. It’s very possible that next CBC may show rebounding neutrophils and platelet counts, and if that’s the case, I’d continue with observation with serial CBCs. However, if the next CBC shows a convincing downward trend of neutrophils and platelet counts, then may consider repeating bone marrow biopsy to determine the etiology and to assess whether hairy cell leukemia is contributing to low counts. So the next laboratory evaluation will tell us more, and please feel free to reach out to us with results and questions at that time.

Question: My brother has been diagnosed few days ago to have HCL. He started yesterday following treatment: 1. Cladribine 10 mg s.c. for 6 days; 2. Allopurinoll tbl 2×1 for 10 days; 3. Glucosalini 1000ml i.v; 4. Sol. Nystatin 3×35 drops 10days; 5. Aciklovir tbl 200mg 2×2 3 days a week; 6. Mycoseb tbl 2×1 3 days a week. Can I have comment on these treatment? I heard that NEULASTA injection can be good addition to boost his white blood cell count after treatment. Can I get comment?

Answer: The role of growth factors in the treatment of hairy cell leukemia is unclear. Studies that have used filgrastim (Neupogen) have yielded inconsistent results, and as such there is no standard recommendation to add growth factors to cladribine in the initial therapy of hairy cell leukemia. The National Comprehensive Network Guidelines for the treatment of hairy cell leukemia also do not mention that growth factor support is necessary. We have used growth factors (typically peg-filgrastim or Neulasta) in patients who are elderly (defined as >65 years), or those who have a number of co-morbidities, or in those who have a past history of radiation therapy or chemotherapy for another cancer, since we would expect them to be cytopenic for a longer duration of time. Please bear in mind that this is not evidence-based, and either approach is acceptable.

Question: I started HCL treatment with Cladribine 8 weeks ago on April 26, 2015. My RBC, HMG and PLT have recovered and are close to standard range now, but the WBC has decreased to 1.2 and Neutrophil to 25%. Is this downward move natural or could it be a sign for unsuccessful treatment? My blood count was as follows. On the first day of treatment: WBC: 6.9 Neutrophils: 37% HGB : 8.5 RBC: 2.71 PLT: 115; Day 7 (the last day of 7 consecutive days) of treatment: WBC: 2.2 Neutrophils: 73% HGB : 10.9 RBC: 3.60 PLT: 133; 8 weeks after treatment began (now) WBC: 1.2 Neutrophils: 25% HGB: 12.5 RBC: 4.36 PLT: 131

Answer: It is very common for neutrophils to be low for some time after CDA. The fact that the hemoglobin is better is very encouraging. I think we should be patient and follow the blood counts carefully. It is very unusual for hairy cell to be resistant to cladribine first line.

Question: Hi. My father 60 years, was given 5-day Cladribine from March 30-April 3. His blood counts almost after 2 months are around TLC- 2000, Hg- 8.9, Platelets- 52000, RBC-2.9. (As soon as we give the growth factor Grafeel his counts shoot up to 2000+). This is the first time after treatment that we stopped grafeel a week ago and he is still maintaining his count. Although his overall condition improved since the treatment (eating normally, walking moderately) he is still getting fever around 100-102 everyday at 12-13 hour intervals for which he is taking Paracetamol. We are in constant watch for other infections but upon repeated tests he was -ve for most bacterial/viral/fungal infections. In between week 6-7 he had no fever for about 3-4 days and completely normal. How many weeks should we wait before the fever completely subsides? Thank you!

Answer: I think that fever should not be present after so many days from end of treatment and I also worry for the persistent cytopoenia. I think that he should continue all prophylaxis and repeat a bone marrow to see what is going on. This is one of the cases when I would recommend a trephine NOT to assess response but to measure toxicity/resistance/ other problems.

Question: I am a 40 year old female. I was first diagnosed with HCL in 2010, then a second time in 2012. It appears that my blood counts are dropping and looks as if my HCL is on it’s way back for a 3rd time. I have not had a recent bone marrow biopsy, but my counts are WBC 3.2, RDW 14.8, PLT 182, GRAN 1.4. I was treated the first two times with Cladribine. I have just learned of the BRAF V600E mutation, but have not been tested for it. My Oncologist isn’t sure what the next treatment will be. Any ideas?

Answer: Unfortunately, this is the problem with HCL that was ignored for many years. That is young patients like yourself being give cladribine with expectation of long-term response only to have relapses fairly quickly within a couple of years. Clearly, this is not satisfactory for you or patients like you. This is why we started the program of chemo-immunotherapy combining cladribine with rituximab for the frontline management of patients with HCL and I do believe that the combination is superior despite the lack of absolute proof in the form of a large randomized trial. Fortunately, we now have multiple options for relapsed disease which are mainly available in the clinical trials that are open at various centers.
Here are the available options:
1. Clinical trial of a BRAF inhibitor such as vemurafenib; we also have the combination of a BRAF and a MEK inhibitor which is thought to be more effective (both oral agents).
2. Clinical trial of a BCR receptor inhibitor, ibrutinib (oral agent).
3. Clinical trial of Moxetumomab which is an antibody targeted against a molecule on the surface of hairy cells.
4. Last, but not least, combined therapy with either pentostatin + rituximab or cladribine + rituximab. This can be done without participation in a trial as long as your insurance company is willing to support it.
5. There are other options like Bendamustine + rituximab.

Question: I am a patient with hairy cell leukemia. My bone morrow is 70% to 80% hairy Cells and my blood counts are low. I have no other symptoms other than fatigue & some tiredness. My Hematologist/Oncologist has suggested treatment with Rituximab once a week for four weeks. Do you have any information on this treatment and its success.

Answer: Most investigators believe that the treatment of choice in general for newly diagnosed patients with HCL is a purine analog. This includes either cladribine or pentostatin. Many prefer cladribine due to the fact that is is only a single 7-day infusion for most patients and the complete remission rate is quite high, on the order of 85%. Rituximab has been studied primarily in the relapsed setting where the complete remission rate is less than half what it is for cladribine among patients who are receiving the drug for the first time. In general Rituximab is reserved for patients who have already been treated with cladribine or pentostatin twice before. Sometimes Rituximab is given with or following cladribine for relapsed disease.