May 2010

A family member is starting Cladribine treatment this week. Is there an average estimated time frame as to how soon after the Cladribine treatment will an enlarged spleen go down?

Resolution of the enlarged spleen in hairy cell leukemia is highly variable. The enlarged spleen usually becomes clinically impalpable between 2 to 4 months. In some patients, a mildly enlarged spleen may persist. This in and of itself is generally not an indication for treatment as long as the blood counts have improved.

When I was first diagnosed 13 years ago, my doctor explained that the HCL cells were infiltrating my spleen and possibly my liver, blocking blood vessels all over my body and causing severe trombocytopenia. That explained the hemorrhages, chronic fatigue, muscle weakness, acute abdominal pains, and peripheral circulatory problems. After a first course with Cladribine I remained HCL-free for 3 years. Then I was diagnosed as a relapsed patient and had a second course of the med. Three years ago I started feeling lethargic and weak and developed osteoarthritis. Since my doctor had told me my blood counts would never go back to normal (they haven't), due to scarified tissue in my bones that would impair normal bone marrow levels in my body, I wonder if there is any connection between these two conditions. Could the HCL have made my bones more susceptible to arthritis?

The symptoms that you describe (in particular lethargy and weakness) may be related to your blood count. You stated that the counts have not returned to normal. It is very difficult to provide an opinion without knowing the actual current counts and the relationship to treatment. Hairy cell leukemia does infiltrate the organs that you listed. The leukemia may cause fibrosis (or scarified tissue) in the bone marrow. However, if the leukemia responds appropriately, the counts should improve if a second remission has been achieved. If a remission is achieved, we sometimes see regression of the fibrosis. If cladribine was unable to produce a second remission, there may be other options that your physician can consider. We have listed a number of contact physicians who may be able to help if your physician wishes to discuss these options.

With respect to “arthritis”, this may be related to hairy cell leukemia. Hairy cell leukemia has been associated with certain auto-immune complications including arthritis. While this may not be typical osteoarthritis, painful swelling in the joints can be associated with the hairy cell leukemia. This may represent another opportunity to discuss these unusual manifestations of the disease. In the web site, we did not extensively cover the rare association of auto-immune phenomena associated with this disease. We are planning to add references relating to this relatively rare complication of the disease.

Greetings - my father 62 was recently diagnosed with HCL upon admission to the hospital for pneumonia. It has been a tough week. He has improved but, lung function has lagged and CT scans and Xrays show something in the lungs.
Yesterday a VATS lung biopsy was performed and thankfully we made it thru however, so far no positive results for infection, fungus or anything. So, is it possible that the "something" in the lungs could be HCL cells collecting there the same way they do in the spleen and then causing these issues? Any help would be appreciated.

We are pleased that your father is improving. It is difficult to accurately answer your question without knowing more of the facts from his physician. Hairy cell leukemia does make patients more susceptible to pneumonia, and sometimes it is very difficult to identify the exact causative organism. Patients can have bacterial pneumonia, or other infectious organisms can be involved. It is appropriate that your physician is attempting to identify the cause. There are a number of other potential causes for lung abnormalities.

In specifically addressing your question, it is possible that hairy cell leukemia can involve the lung. Hairy cell leukemia has been reported to involve the lung in rare case reports.

If your father is improving, it may be that he is responding to the treatment that the physicians are providing for pneumonia. Sometimes, it takes longer to recover from infection in the lung because of low blood counts. When patients with hairy cell leukemia have an active infection, it is important to consider treatment options very carefully. There are a number of institutions listed on this website in our Centers of Excellence section that provide contact points for physicians with questions, or you can always submit questions here in the mailbag.

I was diagnosed a week before Christmas 2009 and received 5 consecutive days Cladrabine 2nd week January 2010. Admitted to hospital 2 days later as I developed a fever and remained in hospital for 3 weeks. Since then I have remained on Aciclovir and every 4 weeks on Pantamadine (IV). I lack energy and lethargy. I believe my last treatment of Pantamadine is next week and will continue Aciclovir for a further 3 months. How long will it be before I regain my energy levels as I think depression is setting in perhaps as a result of frustration not being able to talk to anyone else who has experienced this disease.

Your energy level may return to baseline after the blood counts have recovered from the combination of the disease and the treatment. Sometimes the response is delayed. It would be good to discuss your excessive fatigue with your physician to make certain that your blood counts are responding appropriately. If the blood counts have not adequately recovered, it would be important for the physician to be certain that other causes are not involved. If the blood counts have returned to normal levels, and your excessive fatigue persists it would still be important to discuss this with your physician.

If frustration and depression are setting in, it would also be important to discuss these concerns with your health care team. Having a rare disease can be very difficult, and you may be able to contact your local Leukemia and Lymphoma Society office to see if they have information that may help you. We are not able to provide names of patients who have this illness, but you may find information on the other sections of this website and in the HCL Forum Community that may be helpful.

My wife is currently undergoing radium treatment for DCIS (ductal carcinoma in situ) which was first diagnosed and has just received her third dose of Pentostatin for Hairy Cell Leukemia. Her blood counts have remained very low and do not appear to be improving. The question we have is whether both treatments being carried out concurrently are likely to suppress her blood counts to dangerously low levels?

The combination of localized radiation for DCIS and pentostatin may be contributing to lowering of the patient's blood counts. Both the localized radiation and pentostatin independently might also lower the counts, so that concurrent therapy may add to the lowered counts.

Patients with hairy cell leukemia have low blood counts before therapy because of leukemic cell infiltration in the bone marrow. Pentostatin is a highly effective agent for hairy cell leukemia. However, the initial doses may be associated with temporary worsening of the blood counts. The same response with lowering of blood counts can happen following cladribine for treating leukemia. When treatment is first initiated with either anti-leukemic agent (i.e., pentostatin or cladribine), there can be worsening of the baseline blood counts before there is improvement. In general, the intermittent treatment with pentostatin allows changes and modifications of the dose and schedule to avoid dropping the blood counts to dangerously low levels. The administration of cladribine is often delivered with the single course of therapy over five to seven days. Patients may need to be watched carefully with either drug in case of fever or infection.

Because the therapy is relatively early in the course of treatment, the blood counts may be reduced because of the pentostatin or the radiation or the combination. It would be important to understand the course of events, and to know the doses that are being used. It is possible that the physician involved with her treatment may be able to devise a way to accomplish both goals of treating the hairy cell leukemia and the DCIS. However, it may be necessary to discuss the timing of the treatments and the dose of the drug being used. Because this is a rather complicated case, it might be easier for the physician to contact one of the centers that deals with hairy cell leukemia to discuss the options.

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