After several months of symptoms and numerous doctors visits, I was diagnosed with Hairy Cell Leukemia (HCL) this past Friday (3/27/2009).
As far as leukemia goes, HCL has a good prognosis. 80% of patients go into complete remission after standard chemotherapy (Cladribine), and relapse usually doesn't occur for anywhere from 3 to 12 years. 10% go into partial remission and 5% don't respond. Two other standard therapies include Pentostatin (another chemo) and interferon. Rituximab and BL22 are also being used, so there are lots of approaches available should one not do the job.
HCL is an uncommon neoplastic (malignant) disorder of B lymphocytes (a type of white blood cell) that afflicts middle-aged men. The patient usually presents with pancytopenia (broad spectrum reduction in blood counts -- low platelets, low white blood cells, low red blood cells). There are 500 to 800 cases of HCL annually, representing just 2% of all leukemias. Nevertheless, HCL has been highly researched because it is very treatable and acts as a research catalyst for finding drugs to treat more difficult diseases.
When I went in for my physical in October, 2007, my doctor noted that my platelets were low and that I should check back in 6 months. Work got really busy and even though I was feeling lousy and irritable (dizziness, nose bleeds, insomnia), I attributed it to overwork and poor diet and put off going back to the doctor until this February. Often, I would discuss how lousy I felt with Christi and how I should go to the David Drew Clinic and get checked out head to toe. We had family photos taken in November, and when I saw how gaunt I looked, I knew there was a something wrong. She hung one of the pictures in the hallway and I commented "I hate that picture, it makes me look like I have cancer," but I kept putting off going to the doctor because I didn't think I had the time. Stupid.
I tried to schedule a Dr.'s appointment in December using their on-line appointment service, but they kept calling my home phone instead of my cell to schedule it even though I told them to call my cell phone. After I started feeling dizzy more and more often while sitting down, I finally took the half-hour to wait on the phone, speak to a human being and schedule the appoinment. My regular doctor wasn't available for another 6 weeks, so I scheduled to see a new doctor who was available in 10 days (early February).
At the February physical, CBC results were not good. My platelets were below normal (118) and my WBC was at the low end of normal. My doctor told me to come back in 3 weeks and test again. Needless to say, I stopped working so hard and put my health and family back into perspective.
After 3 weeks, my Dr. ran a differential blood test which confirmed I had thrombocytopenia and absolute neutropenia (low neutrophils -- the white cells that are the first line against infection). Needless to say I was freaking out doing my own differential diagnosis. The hematologist my doctor recommended wasn't available to see me for 5 weeks, so I found another and I'm glad I did. He saw me in 10 days, drew lots of blood and scheduled a follow up appointment for 3 weeks. During that time, he examined my blood and found some characteristics which led him to run a panel for leukemia.
Within a week, his office called to move up my follow-up appointment to discuss what they found. The day after my birthday, I met with him and he presented the results. The phenotype indicated HCL. He did a great job in delivering the news and letting me understand that a BM biopsy was needed to confirm the Dx and that if it was HCL, the prognosis is good and what the treatment would be.
While I ran over to my endodontist to fix a root canal that my dentist completely botched (actually he never found that I had a second dead tooth behind the one he treated) and had remain infected for two months while he just gave me antibiotics, my HemOnc went out of his way to schedule me for a bone marrow biopsy later that morning. I returned to his office and got it done. It wasn't bad. The most painful part was when the needle was removed. The initial tap was dry (typical for HCL) so he went back in with a needle to extract the marrow. I rested, watched the tech prep the slides (marrow is like jelly), then went home for the rest of the day. My doctor also ordered a CT scan with contrast to check for splenomagoly (enlarged spleen), which is a typical symptom of HCL, and any signs of lymph node enlargement (which might indicate lymphoma or other cause). I had the CT scan done the following Wednesday.
Concurrent to these events, I found that I had developed gluten intolerance. For many years, I noticed that typically after eating, I experienced dizziness and itchiness. Likewise, my state of mind was like an emotional roller coaster -- lots of anxiety, paranoia and irritability. My digestion habits were a complete mess and had been for several years. Upon switching to a wheat-free diet, most of my gastro-intestinal problems went away (volume and frequency decreased, no more itchy skin, diarrhea, etc), and my anxiety has ceased to exist. It's made dealing with the HCL much easier.
After the bone marrow biopsy, I started researching HCL in detail, and sent an email to NIH study nurses regarding trials for HCL. I received a prompt reply from Dr. Robert Kreitman, Chief of the Clinical Immunotherapy Section at NIH, regarding a new trial which combines the standard chemotherapy with a biological therapy using the monoclonal antibody Rituximab. I believe the ultimate goal of this treatment is to eradicate/cure the disease -- wiping out every instance of the hairy cells so they can no longer reproduce. The objective stated in the trial summary is "To determine if cladribine and rituximab, whether given together or with rituximab given 6 months after cladribine, is effective in treating residual hairy cell leukemia (disease that remains after the original treatment)."
(My rambling thoughts and overanalysis:) I think this trial is based on the theory that hairy cell starts as a single mutation to one cell which then proliferates exponentially over time. Regardless, if all hairy cells are killed, the body won't produce more unless it is again exposed to mutanagenic toxins (organic solvents like Xylene) and another cellular mutation occurs. Thus, if your hairy cells grow at a known rate before the trial, then one can expect a certain population density to exist in the marrow after several months if residual disease still exists after therapy. If no cells are found after several months, then the disease may in fact be eradicated/cured. I'll ask Dr. K if any of this is correct if he's willing to entertain me on the subject and update or delete it accordingly. Needless to say, I won't be photo-etching my own home-brew circuit boards ever again, and I'm glad I moved from my old house where perchlorethylene (PCE) had leached into the well water (probably from the Texaco up the street dumping degreasing agent into the ground). The county found this after I moved and never bothered to tell me. Nice, eh? At this point, it doesn't really matter how I got it. What's important is that I mitigate or eliminate it.
As you now know, the bone marrow biopsy confirmed the HCL Dx. I'm going to NIH on Tuesday to draw blood that will verify whether I qualify for the trial, discuss concerns and the overall schedule. The most important test will be to see if and how quickly my hairy buggers clone themselves. This is necessary to confirm eradication as the study progresses. If they can't fit me in within 3 weeks, I'll procede with standard Cladribine therapy with my HemOnc.
I'll keep you updated as things move along. My next post will likely discuss the details of chemotherapy and nutrition to slow the cancer and keep it at bay. If nothing else, this has been a wakeup call to eat right, prioritize my life and take better care of myself.
By the way, most of the early research on HCL was done at Ohio State University, starting with its breakthrough discovery in 1958 by Dr. Bertha Bouroncle. My dad would be very proud. Go Buckeyes!
As far as leukemia goes, HCL has a good prognosis. 80% of patients go into complete remission after standard chemotherapy (Cladribine), and relapse usually doesn't occur for anywhere from 3 to 12 years. 10% go into partial remission and 5% don't respond. Two other standard therapies include Pentostatin (another chemo) and interferon. Rituximab and BL22 are also being used, so there are lots of approaches available should one not do the job.
HCL is an uncommon neoplastic (malignant) disorder of B lymphocytes (a type of white blood cell) that afflicts middle-aged men. The patient usually presents with pancytopenia (broad spectrum reduction in blood counts -- low platelets, low white blood cells, low red blood cells). There are 500 to 800 cases of HCL annually, representing just 2% of all leukemias. Nevertheless, HCL has been highly researched because it is very treatable and acts as a research catalyst for finding drugs to treat more difficult diseases.
When I went in for my physical in October, 2007, my doctor noted that my platelets were low and that I should check back in 6 months. Work got really busy and even though I was feeling lousy and irritable (dizziness, nose bleeds, insomnia), I attributed it to overwork and poor diet and put off going back to the doctor until this February. Often, I would discuss how lousy I felt with Christi and how I should go to the David Drew Clinic and get checked out head to toe. We had family photos taken in November, and when I saw how gaunt I looked, I knew there was a something wrong. She hung one of the pictures in the hallway and I commented "I hate that picture, it makes me look like I have cancer," but I kept putting off going to the doctor because I didn't think I had the time. Stupid.
I tried to schedule a Dr.'s appointment in December using their on-line appointment service, but they kept calling my home phone instead of my cell to schedule it even though I told them to call my cell phone. After I started feeling dizzy more and more often while sitting down, I finally took the half-hour to wait on the phone, speak to a human being and schedule the appoinment. My regular doctor wasn't available for another 6 weeks, so I scheduled to see a new doctor who was available in 10 days (early February).
At the February physical, CBC results were not good. My platelets were below normal (118) and my WBC was at the low end of normal. My doctor told me to come back in 3 weeks and test again. Needless to say, I stopped working so hard and put my health and family back into perspective.
After 3 weeks, my Dr. ran a differential blood test which confirmed I had thrombocytopenia and absolute neutropenia (low neutrophils -- the white cells that are the first line against infection). Needless to say I was freaking out doing my own differential diagnosis. The hematologist my doctor recommended wasn't available to see me for 5 weeks, so I found another and I'm glad I did. He saw me in 10 days, drew lots of blood and scheduled a follow up appointment for 3 weeks. During that time, he examined my blood and found some characteristics which led him to run a panel for leukemia.
Within a week, his office called to move up my follow-up appointment to discuss what they found. The day after my birthday, I met with him and he presented the results. The phenotype indicated HCL. He did a great job in delivering the news and letting me understand that a BM biopsy was needed to confirm the Dx and that if it was HCL, the prognosis is good and what the treatment would be.
While I ran over to my endodontist to fix a root canal that my dentist completely botched (actually he never found that I had a second dead tooth behind the one he treated) and had remain infected for two months while he just gave me antibiotics, my HemOnc went out of his way to schedule me for a bone marrow biopsy later that morning. I returned to his office and got it done. It wasn't bad. The most painful part was when the needle was removed. The initial tap was dry (typical for HCL) so he went back in with a needle to extract the marrow. I rested, watched the tech prep the slides (marrow is like jelly), then went home for the rest of the day. My doctor also ordered a CT scan with contrast to check for splenomagoly (enlarged spleen), which is a typical symptom of HCL, and any signs of lymph node enlargement (which might indicate lymphoma or other cause). I had the CT scan done the following Wednesday.
Concurrent to these events, I found that I had developed gluten intolerance. For many years, I noticed that typically after eating, I experienced dizziness and itchiness. Likewise, my state of mind was like an emotional roller coaster -- lots of anxiety, paranoia and irritability. My digestion habits were a complete mess and had been for several years. Upon switching to a wheat-free diet, most of my gastro-intestinal problems went away (volume and frequency decreased, no more itchy skin, diarrhea, etc), and my anxiety has ceased to exist. It's made dealing with the HCL much easier.
After the bone marrow biopsy, I started researching HCL in detail, and sent an email to NIH study nurses regarding trials for HCL. I received a prompt reply from Dr. Robert Kreitman, Chief of the Clinical Immunotherapy Section at NIH, regarding a new trial which combines the standard chemotherapy with a biological therapy using the monoclonal antibody Rituximab. I believe the ultimate goal of this treatment is to eradicate/cure the disease -- wiping out every instance of the hairy cells so they can no longer reproduce. The objective stated in the trial summary is "To determine if cladribine and rituximab, whether given together or with rituximab given 6 months after cladribine, is effective in treating residual hairy cell leukemia (disease that remains after the original treatment)."
(My rambling thoughts and overanalysis:) I think this trial is based on the theory that hairy cell starts as a single mutation to one cell which then proliferates exponentially over time. Regardless, if all hairy cells are killed, the body won't produce more unless it is again exposed to mutanagenic toxins (organic solvents like Xylene) and another cellular mutation occurs. Thus, if your hairy cells grow at a known rate before the trial, then one can expect a certain population density to exist in the marrow after several months if residual disease still exists after therapy. If no cells are found after several months, then the disease may in fact be eradicated/cured. I'll ask Dr. K if any of this is correct if he's willing to entertain me on the subject and update or delete it accordingly. Needless to say, I won't be photo-etching my own home-brew circuit boards ever again, and I'm glad I moved from my old house where perchlorethylene (PCE) had leached into the well water (probably from the Texaco up the street dumping degreasing agent into the ground). The county found this after I moved and never bothered to tell me. Nice, eh? At this point, it doesn't really matter how I got it. What's important is that I mitigate or eliminate it.
As you now know, the bone marrow biopsy confirmed the HCL Dx. I'm going to NIH on Tuesday to draw blood that will verify whether I qualify for the trial, discuss concerns and the overall schedule. The most important test will be to see if and how quickly my hairy buggers clone themselves. This is necessary to confirm eradication as the study progresses. If they can't fit me in within 3 weeks, I'll procede with standard Cladribine therapy with my HemOnc.
I'll keep you updated as things move along. My next post will likely discuss the details of chemotherapy and nutrition to slow the cancer and keep it at bay. If nothing else, this has been a wakeup call to eat right, prioritize my life and take better care of myself.
By the way, most of the early research on HCL was done at Ohio State University, starting with its breakthrough discovery in 1958 by Dr. Bertha Bouroncle. My dad would be very proud. Go Buckeyes!
