A patient’s point of view

EARL J. STONE

Hairy Cell Leukemia Research Foundation, Inc., Deerfield, IL, USA.

AbstractPatients have many concerns when diagnosed with hairy cell leukemia (HCL). Their ultimate concern is whether they will becured. Although purine nucleoside analogs have been very effective in the treatment of HCL, some patients do not respondto these therapies and many relapse after initial treatment. From a patient’s point of view, it is gratifying to know thatresearchers continue their efforts to develop more effective treatments with minimal side effects. By forming the Hairy CellLeukemia Consortium, these researchers seek to educate patients and doctors around the world and continue their quest fora cure for hairy cell leukemia.

Keywords: hairy cell leukemia, survivor

As President of the Hairy Cell Leukemia Research

Foundation and a hairy cell survivor, I was invited to

speak from the patient’s point of view at the 2010

international hairy cell leukemia symposium on the

Treatment of Hairy Cell Leukemia in its Second

Half-Century. I want to thank the Hairy Cell

Leukemia Consortium and the National Cancer

Institute for giving me the opportunity to speak to

an esteemed group of researchers on behalf of myself

and other survivors of hairy cell leukemia.

In 1998, I was 44 years old and an avid runner

when a routine blood test showed that my platelet

count was low. After ruling out other possible

diseases, my hematologist did a bone marrow biopsy.

Several days later, my hematologist called. He said, ‘I

have good news and bad news. The bad news is that

you have leukemia. The good news is that you have

hairy cell leukemia.’ I had never felt sick, and all of a

sudden I was being told I was sick—and it was

cancer.

Once the initial shock wore off, I wanted to learn as

much as I could about hairy cell leukemia (HCL). I

called the National Cancer Institute, Leukemia &

Lymphoma Society, American Cancer Society, and

the Hairy Cell Leukemia Research Foundation to

request information on HCL. At my local hospital

medical library, I read hematology textbooks and

every published article I could find on HCL. All this

information led me to three doctors, whose names

and institutions I repeatedly found in the research

materials.

Consulting with all three of these HCL specialists,

as well as their pathologists, gave me more good news

and bad news. The good news was that I had hairy

cell leukemia. The bad news was that while each

doctor recommended I be treated with cladribine (2-

CdA), each had a different opinion as to when I

should begin treatment. Some recommended I be

treated immediately; others thought I should not

start treatment until my blood counts dropped

further. While all the doctors had good reasons for

their recommendations, after numerous discussions

with my hematologist and my wife, I decided to start

cladribine while my blood counts were still good and

I was feeling strong. I hoped I would weather the side

effects of cladribine better than if I waited until my

counts fell lower. Plus, I felt that I just needed to do

something—for me, watching and waiting just was

not going to work. This was a good choice for me. I

took care of myself, did what I was capable of doing,

went for my blood tests, and got through the

treatment. My counts recovered almost exactly as

they did for the patients in the 2-CdA clinical trials I

read about in my research.

Correspondence: Earl J. Stone, President, Hairy Cell Leukemia Research Foundation, Inc., Deerfield, IL, USA. E-mail: hairycellleukemia@hotmail.com

Leukemia & Lymphoma, June 2011; 52(S2): 108–109

ISSN 1042-8194 print/ISSN 1029-2403 online � 2011 Informa UK, Ltd.

DOI: 10.3109/10428194.2011.573035

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Despite having extremely hypocellular bone mar-

row and suppressed CD4 counts, my follow-up bone

marrow biopsy looked good. I continued to see my

hematologist regularly and tried to take better care of

myself. However, less than 3 years after my treatment

with cladribine, I relapsed. Again, I did not feel sick.

Though my blood counts were falling, the percentage

of hairy cells found in the bone marrow was small. I

spent the next several months doing more research

and consulting with more HCL specialists. Having

very hypocellular bone marrow and being CD25

negative, I received more conflicting opinions on

treatment options: Rituxan for 4 weeks? The BL22

clinical trial at NCI? Cladribine again? Interferon?

Rituxan for 8 weeks?

Again, working with my hematologist and follow-

ing my gut feeling, I chose the 8 weeks of Rituxan

protocol. While I knew I could participate in a

clinical trial, I elected to be treated at home by my

hematologist because I did not want to travel for

treatment. Other than having suppressed B cells for

several years following the Rituxan treatment, and a

cardiac arrhythmia that I feel may have been related

to Rituxan, it was the best decision I ever made.

Eight years later my blood counts are perfectly

normal.

Since the time of my diagnosis, I have been

involved with the Hairy Cell Leukemia Research

Foundation. The Foundation is an all-volunteer

organization formed more than 25 years ago to raise

funds for HCL research and to provide a network of

support for patients and their families from around

the world.

Through the Hairy Cell Leukemia Research

Foundation, I often hear other patients’ concerns in

dealing with hairy cell leukemia. While each patient’s

situation is unique, based upon my experience and

conversations with other patients, I believe that all

patients with HCL have similar concerns:

. Is the treatment my doctor recommended the

best treatment?

. My doctor has never treated anyone with hairy

cell leukemia; who are the best HCL doctors?

. What causes hairy cell leukemia?

. Is hairy cell leukemia hereditary?

. What are the side effects of the treatment?

. Are there any long-term side effects of the

treatment?

. When should I have a follow-up bone marrow

biopsy and computed tomography (CT) scan?

. Should I begin treatment now, or wait until my

blood counts get worse?

. What are the chances that my hairy cell leukemia

will return?

. Can I prevent a relapse by taking vitamins?

. Am I going to die from hairy cell leukemia?

After listening to the speakers at the symposium

discuss their research, I am confident that one day we

will have answers to all of these questions.

When I was diagnosed in 1998, purine nucleoside

analogs were the latest and greatest and seemed to

work for most patients. However, follow-up studies

showed that some patients do not respond to these

therapies and many eventually relapse after their

initial treatment [1]. While I am excited about the

future of hairy cell leukemia research, further

research into the biology of HCL and a better

understanding of minimal residual disease is neces-

sary to find a cure for hairy cell leukemia. In addition,

since hairy cell leukemia is a rare disease and most

patients are not treated as part of a clinical trial, it is

important that statistical data (such as age, gender,

occupation, treatments, ethnicity, etc.) be gathered

to allow researchers to find common characteristics

among patients with HCL. Finally, creation of a

tissue bank may help researchers develop even more

effective treatments with minimal side effects for

hairy cell leukemia.

Despite all the advances in the treatment of hairy

cell leukemia during the past 50 years, the research-

ers who have dedicated their lives to finding a cure

for HCL are not satisfied. By forming the Hairy Cell

Leukemia Consortium, they are continuing their

quest for a cure. These researchers will work together

to educate doctors around the world about HCL,

encourage young doctors and scientists to go into

HCL research, and discover the causes of HCL so

that one day hairy cell leukemia will be completely

eradicated.

Potential conflict of interest: A disclosure form

provided by the author is available with the full text of

this article at www.informahealthcare.com/lal.

References

1. Grever MR. How I treat hairy cell leukemia. Blood

2010;115:21–28.

2. Holzman D. Has success spoiled hairy cell leukemia research?

Key questions go unanswered, despite big gains. J Natl Cancer

Inst 2009;101:370–373.

HCL Meeting 2010 109

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