July 2010

I was diagnosed with HCL in October of 2007 and treated with 2CDA. At the time of treatment my platelets were 90,000 and white counts were very low. Bone marrow biopsy showed no hairy cells 6 months after treatment and my platelets returned to normal and never went higher than the 170's. In July of 2009 my platelet count was 175,000. A emergency room visit for severe fatigue and shortness of breath in Sept of 2009 showed my platelets had dropped to 148,000 and the doctor wanted to watch and wait and repeat labs every 3-6 months. In February of 2010 my platelets dropped to 116,000 and in April of 2010 went back up to 143,000.

Summary:
July 2009 Platelets 175,000
Sept 2009 148,000 E.R. Visit fatigue and SOB.
Feb 2010 116,000 pain in spleen area started.
Lab repeated one week later to check for lab error and platelets were 123,000.
April 2010 platelets back up to 143,000.

This is how my thrombocytopenia presented at the time of diagnoses but my white counts were also low. Are these platelet count fluctuations consistent with the HCL being active again? If so, why would the platelets be going up and down with a 50,000 variation in a six month period? HG/HCT and white cells are all still normal. Should a bone marrow biopsy be repeated? This service is so helpful and I thank you for the expert opinion.

It is very gratifying that you have had a great response to the Cladribine. It was important that you had the follow-up bone marrow biopsy documenting that the hairy cell leukemia was in remission. Achieving a complete remission is the goal. From your note, you indicate that your platelet count returned to normal. You also stated that the white blood cell count and the hemoglobin and hematocrit are normal.

Some fluctuation in the platelet count is normal. In following patients after effective treatment, we do initially repeat the bone marrow to confirm a complete remission. However, we do not do regular routine bone marrow studies unless there is clinical suspicion that a relapse is present that requires consideration for re-treatment. In our experience, we do repeat the bone marrow before re-treatment to make certain that we understand the reason for the falling blood counts.

In patients who are in complete remission, we will still be able to identify residual hairy cell leukemia by special studies on the bone marrow in a number of patients. We do not routinely re-start therapy unless there is a sustained fall in the blood counts. There is considerable clinical judgment regarding when patients will need to be re-treated. We do not want to re-treat patients too soon to avoid undue side effects. However, we also do not want to delay re-treatment until the counts have fallen too far. So, this is where the hematologist will often follow the blood counts every three to four months to get a sense of the course of the disease. Incorporating the symptoms and the physical findings of spleen size are all important factors that your doctor will use in helping to make these decisions.

I was diagnosed with HCL in April 2010. My white blood count is low (1900) but my red blood count (3.82) and platelets ((177) are within normal range. I've been feeling extremely tired,weak and achy. Do you recommend I start treatment now or wait until my WBC and platelets drop?

After making the correct diagnosis of hairy cell leukemia, the next important question will be when to start therapy. There are published recommendations that are based upon the opinions of practicing hematologists. It is not possible for us to make a specific recommendation about when to start your individual treatment. This is a decision that your physician will need to make with your involvement. In general, patients who are having symptoms that warrant treatment will have a progressive decline in their blood counts. Patients are often followed with routine blood count determinations to watch the trend and to see if symptoms or signs develop that require treatment.

There has been a “watch and wait” approach following the patient and the blood counts in making these decisions. Because the treatment may have side effects, the decision of risk versus benefit needs to be explained to the patient. The physician may decide to not start treatment if the patient is not having symptoms until there is a consistent trend that the blood counts are declining. However, it is also important to start therapy before the blood counts have dropped to a dangerous level. Therefore, your doctor will need to review his plan with you.

I was diagnosed with HCL in 2003: Cladribine induced remission. I have had 2 doses of Basal Cell cancer - 1 before, the other after - but I am now finding that I get far more skin blemishes - tags, moles, 'horny sickles' etc. I do not sunbathe: I avoid the sun. Is this a factor of HCL?

There have been case reports noting that patients with hairy cell leukemia can develop basal cell cancers and other skin cancers. In general, it is a particularly good policy to practice good cancer prevention and screening. It is wise to avoid “sun bathing” as you already know. It would be good to discuss these concerns with your dermatologist. It may be a good idea to have regular check-ups with your dermatologist to insure that you have careful skin examinations of these skin “blemishes” that you describe. The dermatologist can also provide advice with respect to the best sun screens for the unavoidable normal exposures.

I have a choice to have a 5 day treatment of Cladribine 2 hrs a day for 5 days or in-patient for 7 days of Cladribine 24 hrs a day. This seems like a huge difference in quantity of Cladribine. I don't understand how my oncologist can leave this decision up to me. Do I want to receive 10 hrs of chemo or 168 hrs of chemo? How can I make that choice? I feel this should be my doctors decision. What is your advice on this?? Thanks for your time.

Cladribine is a very effective drug for the treatment of hairy cell leukemia. The original studies used the seven day treatment schedule with the drug being delivered through a continuous intravenous administration. This regimen was initially developed by experts at Scripps. The long-term follow-up results on their studies using this regimen have been published.

Other hematologists have used the interrupted schedule for drug administration with Cladribine. These experts deliver the drug over 1 to 2 hours each day for five days. The long-term follow-up results on those studies have also been published. They believe that the results are equivalent. The actual daily doses of the drug may vary depending upon the protocol that your physician is following. Because this is a rare disease, there has not been a direct randomized controlled study to my knowledge to determine which approach is better.

Mailbags

HCL Forum

Ask an Expert