Depending on whose figures you believe, some 5% to 11% of CLLers will come down with this red-blood-cell-destroying, potentially-life-threatening nonsense. It happened to me early in 2007. (For more details on what it is and how it's treated, read this post. The NIH has a rundown here.)
I've had a bad time of it, a more severe case than most, becoming refractory to the standard treatments: steroids, rituximab, cyclophosphamide. As of 2009 it was getting so bad that I was relapsing -- in the form of severe bouts of hemolysis of red blood cells -- every few months, no matter what treatment I did. Save for a splenectomy, which is no guarantee of long-term success, I was running out of options.
Along came Revlimid. I have discussed AIHA and Revlimid (lenalidomide) in some recent posts, but the information was buried.
I don't want to bury the lede here: I am now Coombs negative and have not had a bout of hemolysis since I began Revlimid in March. In my more giddy moments I wonder if I am cured of AIHA.
Revlimid, an immunomodulator drug, has evidently reset my immune system so that it is no longer making antibodies to my red blood cells, a process that leads to hemolysis, which is when macrophages attack those cells. My red blood cell count, along with hemoglobin and hematocrit, has been normal since shortly after I began Revlimid. The Coombs (aka Direct Antiglobulin Test) I had on Oct. 6 confirms that I am negative for those antibodies.
To the best of my knowledge, the effect of Revlimid on CLL-induced AIHA has not been studied nor reported. I believe it is potentially one of the hidden headlines in today's CLL news. Aside from my anecdotal experience, a leading researcher has also found instances where it has been beneficial. In response to an e-mail I sent, Dr. Asher Chanan-Khan wrote:
"I have some experience in context with AIHA. At least 2 patients treated with len while transfusion dependent due to AIHA, resolved their need for transfusion and at least 1 is alive without AIHA 3+ years. Although the numbers are small, it does suggest that AIHA can be controlled with control of disease with len."
What I am saying here to fellow members of the elite club nobody wants to join is this: Consider Revlimid. I was on 5 mg when progress started being made, so it probably won't require a huge dose to control the AIHA. Revlimid may be useful at the very start of your battle as the side effects are usually less problematic than those associated with the traditional first line of defense, steroids.
There is, of course, no guarantee that Revlimid will work for everyone. There is one report of Revlimid actually inducing warm body AIHA in a woman with diffuse large B cell lymphoma. But that's not CLL.
I also have been on monthly doses of Arzerra during some of this period (ending two months ago). It is possible that the anti-CD20 monoclonal antibody also contributed to my progress, although it appeared to have little effect on the disease and we have now suspended its use. The fact that I was refractory to a similar drug, Rituxan, may explain why Arzerra (ofatumumab) appeared to be providing me with little benefit.
It would be helpful if some researcher somewhere would undertake a study on Revlimid and CLL-induced AIHA; if the results are what I think they will be, Revlimid could become an important tool in the battle to control CLL, reset the immune system, and perhaps put an end to the scourge of AIHA for large numbers of patients.
Today is January 18, 2011. It has now been almost five months since I quit the Arzerra. Still no sign of AIHA or hemolysis, red counts remain in the normal range, nothing out of whack.