Addressing Racial Disparities in a Rare Blood Cancer

Racial disparities in care and outcomes have been well documented but the problems can be particularly acute in cases of rare, genetic diseases. One example of this is the rare blood cancer cutaneous T-cell lymphoma. African Americans are twice as likely as people of European or Asian descent to develop CTCL, are typically diagnosed with more advanced disease, and have a lower survival rate from the condition. Kyowa Kirin North America, which produces the CTCL treatment Poteligeo, is working to address racial disparities to improve the diagnosis, care, and outcomes of African American patients with CTCL. We spoke to Kyowa Kirin Vice President of Public Affairs Lauren Walrath and Co-Leader of the Immune Cell Regulation and Targeting Program at the Sidney Kimmel Cancer Center at Jefferson Health Pierluigi Porcu, about CTCL, the disparities in care and outcomes for African Americans with the condition, and what they are doing to address that. Porcu is a paid consultant to Kyowa Kirin. 

Daniel Levine: Pigi, Lauren, thanks for joining us.
Lauren Walrath: You’re welcome.
Daniel Levine: I’m going to talk about the rare blood cancer cutaneous T-cell lymphoma, how racial disparities in this condition can affect outcomes, and what Kyowa Kirin North America is doing to change that. Let’s start with CTCL, though. Pigi. What is CTCL?
Pierluigi (Pigi) Porcu: So, CTCL is not just one disease, it’s a group of lymphomas—cancers of the blood cells call lymphocytes. Lymphocytes are the main cell of the immune system. So, CTCL is one of the lymphomas of the immune system. Overall, lymphomas can be B-cell and T-cell, and most of the lymphomas in the U.S. are B cells. So, T-cell lymphomas as CTCL are rare diseases.
Daniel Levine: And how does the condition manifest itself and progress?
Pierluigi (Pigi) Porcu: Usually patients develop flat, scaly rash on certain parts of their body. That’s the most common presentation, particularly the abdomen, the back, the armpits, the groin. Those are the most common signs of disease, very slow, very gradual, subtle, sometimes gets better with sunlight exposure. So people would have less rash in the summer and they will have a more visible rash in the winter. Sometimes it’s itchy but not always.
Daniel Levine: The fact that this can present as more common dermatological conditions, how does that complicate getting it in accurate diagnosis?
Pierluigi (Pigi) Porcu: Well, that is a problem, of course, because very often in the early stages CTCL doesn’t look any different compared to other common rashes like eczema, psoriasis, atopic dermatitis, allergies, and so on; so really requires a high level of attention. And unlike a lot of the other rashes, it can tend to progress and become more extensive over time so at some point, the patient goes to the dermatologist, and I think one of the most important things that can be done is to do a skin biopsy. Even if you do a skin biopsy though, in early stages the diagnosis can still be difficult because even under the microscope, the skin can look very similar to more common rashes, not lymphoma. So, it’s a big challenge.
Daniel Levine: How much of a problem is it that patients just ignore the early signs of this?
Pierluigi (Pigi) Porcu: It is a problem. It may not be a huge problem in the sense that eventually with progression of the rash at some point the patient goes to a dermatologist who may be familiar with the disease and have biopsies that eventually show it. But certainly, there is a significant delay in diagnosis and even if the delay is not dramatic in terms of the impact on survival, it still certainly delays the initiation of treatment that can make people better. So, it is a problem.
Daniel Levine: How is the condition treated and what’s generally the prognosis for someone?
Pierluigi (Pigi) Porcu: So, usually when patients present with [what] we call early stage, they have the rash primarily on the skin and the lymphoma is only localized to the skin. The treatment is something that we call skin directed therapy, so primarily topical therapy. It could be steroids; it could be other drugs applied to the skin. It could be ultraviolet light given through the ultraviolet light booth, or sometimes is radiation. That’s called skin directed therapy. When patients have more advanced disease or they go through skin directed therapy first and they continue to have disease, then they have to start something called systemic disease. These are medicines that people have to take by mouth or injection often for an extended period of time. The prognosis for patients who have early stage disease in terms of survival, remember that this is a cancer after all, it’s measured in 10 to 15 years. So the prognosis is very good. But for patients who have advanced stage disease, those who need to take systemic therapy and so on, the prognosis can be significantly worse, and sometimes it can be as little as two to three years survival.
Daniel Levine: What’s the relationship between timely diagnosis and outcomes? How important is it to catch this earlier and its progression?
Pierluigi (Pigi) Porcu: It is important because even if somebody has early stage disease, until you really know the specific diagnosis it is difficult to start therapy that is more specific for CTCL as opposed to any other generic rash. There’s also evidence that for patients who have really delayed diagnosis, the rate of progression is higher and the patients can find themselves having more advanced stage than otherwise they would’ve had if they had made a diagnosis earlier.
Daniel Levine: African Americans are twice as likely to develop CTCL relative to people of European or Asian descent. How does the diagnosis of African Americans compare to others and how do outcomes compare for them?
Pierluigi (Pigi) Porcu: Yeah, so one of the challenges with the diagnosis of CTCLin African Americans is that because of the pigmentation of the skin it’s more difficult to see the rash because the rash typically causes redness. And depending on the light or the level of attention, it may be very difficult to detect the earlier signs of development of the rash. If people have significant amount of itching, then that tends to make it easier to actually catch their attention. Another possibility is that in some African American patients, CTCL can cause loss of pigmentation. So, the skin actually looks a little lighter in color compared to the rest of the skin, and that also brings attention. But in general, there are significant challenges in making an early diagnosis of CTCL in African Americans, and that’s well documented in several studies.
Daniel Levine: How well understood are the causes of the disparities in diagnosis and outcomes? Is this a physician issue? Is it a patient issue? Is it just something that’s specific to the disease in these patients?
Pierluigi (Pigi) Porcu: I think that it’s fair to say that we don’t know for sure because the data is fairly limited; certainly, good quality data are limited. So, I think we’re doing a lot of guessing at this point. Just the physical challenges of visualizing the rash probably plays a role in delaying the diagnosis. Most likely, there’s also an issue of access to healthcare, particularly specialized care with dermatology. I’m sure that that’s part of the problem, and I think that perhaps part of the problem could be that patients tend to go and see the doctor less early as opposed to other patients. So, access to the diagnosis, the physical appearance of the skin, I believe, those are significant reasons why the diagnosis delayed.
Daniel Levine: Lauren, let’s bring you in here. Kyowa Kirin won FDA approval for Poteligeo in 2018. What is it and how does it work?
Lauren Walrath: Our treatment, Poteligeo, is a monoclonal antibody that uses the body’s own immune system to target and eliminate CCR4 cancer cells, which are frequently expressed in hematologic malignancies, including CTCL. Poteligeo is approved for two more common forms of CTCL, and those are called Sézary syndrome and mycosis fungoides. Mycosis fungoides is more common than Sézary syndrome, but Sézary syndrome itself is a more advanced and severe form of CTCL. Poteligeo leverages a proprietary technology that Kyowa Kirin had developed called Potelligent, and Potelligent actually helps enhance the antibody dependent cellular cytotoxicity, so the ability of the medicine itself to bind to the target cancer cells. And so, that is how it works. And we ran a full development program with a pivotal study called the Maverick study and that was the basis for approval here in the U.S.
Daniel Levine: Kyowa Kirin is seeking to raise awareness about CTCL on the black community. How is it going about doing that?
Lauren Walrath: Sure. Well, as we’ve talked about, African Americans are twice as likely to develop CTCL compared to patients of European or even Asian descent, and typically they’re also presenting with more advanced disease. And our history, I think, and really our knowledge of these disparities was enhanced by working with investigators like Dr. Porcu on the Maverick trial. These investigators really help the company appreciate that we really needed to target a very representative patient population with our clinical program and ensure that we collected data on a truly representative patient population so that we would know how the drug worked in African Americans as well as in other patients who are more commonly involved in clinical trials. So, really our awareness of this issue goes all the way back to the clinical program. And now that the drug is approved, we believe we have a commitment and a duty to make sure that we’re raising awareness among the broader patient population as well as among HCPs about the issues that affect this patient population. And that’s part of what we do in the public affairs team that I lead for Kyowa Kirin in North America. We’ve been working now for several years on education initiatives. We have engaged with different healthcare organizations, with different patient advocacy groups, to help spread the word, in particular at certain times of the year when we think there’s more attention being paid to black patient health issues and issues of concern to black populations. So, we are out there trying to work with partners to deliver really good information and content that can help in driving more awareness of this rare disease and of its incidence among the black patient population.
Daniel Levine: In addition to raising awareness, the company launched grant programs to fund work aimed at diminishing racial disparities and improving outcomes. How much money is Kyowa Kirin putting into this effort and what are they specifically doing?
Lauren Walrath: Sure. Our grant program started back in 2020, and the first disbursement of funds amounted to about $120,000, and because we were really pleased to see the response from the different audiences and some of the ideas that were coming into our organization, we re-upped our grant funds and we continue to disperse funds for appropriate proposals that come into the company. We’ve gotten a really amazing, I think, set of ideas from different organizations that are out there. The ideas and the proposals that we’ve funded cover everything from translational research projects to epidemiological studies that really try to look at what are some of the root causes that could be behind some of these disparities. In addition, we have funded a number of education and community outreach programs to help reach the target black patient population in certain cities and certain urban areas, as well as in regions where we believe there is a higher incidence of CTCL among African Americans. Those education programs have been run by patient advocacy groups. They’ve been run by cancer clinics, they’ve been run by large academic hospitals. And so, we’re very proud that we’ve dispersed these funds and we will continue to disperse these funds when we get good proposals and recommendations. To date that’s amounted to about $250,000 over the past two years, and our plans to continue to support good programming and good research projects that will help, hopefully, in raising understanding of the issues behind these disparities will continue into the future.
Daniel Levine: The company is not just focused on patients, but also on physicians and getting them to look for the condition. What is Kyowa Kirin doing to get physicians more attuned to CTCL, particularly with African American patients?
Lauren Walrath: Well, we certainly hope with some of the projects that we’ve supported with our grant funds, that there will be publications and that research will be shared with the wider community. So, we are hopeful for that and we will continue to invest in those activities, but from our own activities that we manage and work on ourselves, we have continued to look for new and different ways to expand our reach and to drive more awareness among the treating physicians. Some of the things we’ve done, we organized a round table discussion that Dr. Porcu was part of this year, and we did that as part of Black Family Cancer Awareness week back in June. And we’ve taken some of the content from that effort and shared it both with Dr.Porcu’s institution, Thomas Jefferson Hospital, as well as with some of the other institutions that joined us for that panel and the advocacy organizations. And so, we’re using as many channels as we can to spread some awareness about the disparities affecting the CTCL patients. In addition to that, our medical affairs teams and our sales reps from Kyowa Kirin who go out there and speak with doctors are definitely attuned to when this conversation is relevant and they have materials and research that they can share, including the data from our Maverick trial that help physicians understand potentially whether Poteligeo may be inappropriate treatment for a black patient or any patient suffering with the two forms of CTCL that were approved for.
Daniel Levine: Pigi, what does your hospital do to reach out to or care for multicultural or black patients? Is there special training you provide doctors? Do you do any outreach with community organizations?
Pierluigi (Pigi) Porcu: Yeah, absolutely. First of all, Thomas Jefferson University or Hospital is really an urban hospital deep in the center city of Philadelphia. So, we have a very large population of patients. We care for a very large population of patients who are black African Americans and a lot of other minorities. There is really a culture of outreach, a preparedness about being culturally knowledgeable, including language availability for non-English speaking people. All the way to the medical school, a lot of interest in training and preparation for that. We partner with patient advocacy organizations both here in Philadelphia as well as nationally. One of them is the Cutaneous Lymphoma Foundation that is the only organization that focuses on patients with cutaneous lymphoma, but also others like Lymphoma Research Foundation, the Leukemia Lymphoma Society, and so on. So there’s a lot of give and take partnerships with all these organizations here in Philadelphia.
Daniel Levine: Lauren, as the company has sought to reach out to the African American community, what has it learned about building trust within this community and how to effectively reach people?
Lauren Walrath: I think the learnings are still ongoing. I think a lot of the feedback we’ve been getting from our events, and in particular some of the live events like the round table I mentioned, is that opening the conversation and helping people learn to self-advocate is one of the most important and powerful messages that we can deliver. We had a wonderful woman who joined us, I think she’s a patient of Dr. Porcu, and she told her story about trying to get care, trying to get treatment. She had visited a dermatologist several different times. She probably visited more than one. She brought along patients and family members to her appointments, trying to ensure that maybe if the doctor wasn’t understanding her and her symptoms and the way she was reporting them, that maybe they would listen to somebody whose accent was a little bit different or who was light skinned and maybe might be treated a little bit differently by the healthcare system. This was one woman’s example, but it was certainly an example of the challenges that patients may face in a system that doesn’t always recognize the disease. And it is a rare disease so it’s not maybe commonly understood and that is part of the hurdle. But I think what we’re trying to do, and working with institutions like Thomas Jefferson University Hospital System, working with the Cutaneous Lymphoma Foundation, working with LRF and the other advocacy groups, is make sure that the information is out there so that these patients when they report symptoms, when they seek care, that they are heard, and hopefully people can help recognize what it is that they’re talking about. I think the feedback that we get is that these patients really do need a more responsive healthcare system and they need assistance sometimes too, from maybe nontraditional healthcare resources like community social workers or pharmacists or people in their local communities. And so, we’re encouraged. I think that hospital systems in urban areas are taking those needs to heart and starting new teams that expand their outreach and hopefully build trust into the local communities. We certainly want to continue to see that and support that where we can. We also want to continue to do what we can to raise awareness and to drive the conversation because we hope that in doing so, ensuring that these patients experiences are heard and understood, but also there will be more people out there willing to listen and when they hear the symptoms being reported that they’ll be somewhat more knowledgeable about how to help that patient access the best care that they can.
Daniel Levine: Pigi, how about your program? What have you learned about effective ways of addressing racial disparities in CTCL and what have you found works? What doesn’t work?
Pierluigi (Pigi) Porcu: Yeah, so I think that one of the most important things, in addition to what Lauren mentioned, which is the outreach and giving people the opportunities to speak up, is to really build a team that is very knowledgeable on this particular disease. These are rare diseases; therefore, a lot of doctors and healthcare providers may see a patient like this once in medical school and then they forget. And even on the dermatology side, there may not be a sufficient familiarity with this. So, I think the important thing is to educate and build a team. Here at Jefferson, we have a multi-d clinic and program, which means that we work very closely together. I’m a medical oncologist, so I’m a lymphoma doctor. I work very closely with my colleagues in dermatology and radiation oncology and pharmacy and social workers. We have a whole program, this is true for a lot of other institutions and is not unique to Jefferson, but it’s more difficult to find that kind of infrastructure in the community. So, in the community, what we do, because of the footprint that Jefferson has, which is a mixture of an academic center in Center City with other community based academic programs across the region, we are deploying a system of lectures, education, in-person conferences to discuss these rare diseases and make everybody familiar with it. But it’s a lot of work and requires education and takes some time.
Lauren Walrath: Just to build on that for one more moment, in our panel discussion back June, it also became quite clear what Dr. Porcu just said, which is that because medical training is done and somebody goes on then to hopefully have a nice career, but they may not see these patients frequently, and it may be decades before they encounter another patient with CTCL. It really is important to continue to fund continuing medical education and to make sure that there is more up-to-date information available for the physician community. One of the things that came out of that roundtable was that there needed to be more examples of CTCL patients and their symptoms on dark skin. That photography, for example, was something that might be missing from some of the medical literature and certainly some of the medical school programs. I was speaking with some members of our medical affairs team recently, and they were telling me that there is a new project underway to make sure that that photography is being captured and that more education programs will be able to leverage that in the future. I think that’s a really good sign too, of what else can be done in terms of educating the physicians, and again, it stems from a good dialogue that’s happening across these organizations, across the healthcare institutions, the opinion leaders like Dr. Porcu, the advocacy organizations, and where Pharma assistance can help.
Daniel Levine: As drug developers and providers have become sensitized to these issues and seek to take steps to address them, what advice would you offer?
Lauren Walrath: Well, I think commitment is key. I mentioned that I think the inspiration for this work and the work we continue to do really stemmed from the clinical program, the Maverick program, and that we had advocacy groups and investigators who were helpful in educating our company about what the real patient population looks like and encouraging us to keep that in mind through the development work we were doing, but also now through the commercial work that we do to support our business. Disparities certainly have arisen in our healthcare system over decades, over centuries, really. And they aren’t rooted in one cause or another. They’re to some degree, as Dr. Porcu started out saying, they’re an issue that comes from many different root causes, but in order to address them and to hopefully see real improvements, you have to stay committed. I don’t think you can imagine that any one program or any one grant is going to solve the problem. It requires a true commitment and a sustained commitment over time to hopefully see improvement.
Pierluigi (Pigi) Porcu: Okay, let me just add one thing to that if I may to expand on the Maverick trial that really kind of led to the approval of the drug. It is very important because then it is giving us the opportunity to go back and look at and ask specific questions about the population that was enrolled on the Maverick, and now we’re starting to actually get some information on that. For example, on the Maverick trial, 10 percent of the patients that were enrolled were blacks, self-identified blacks so we were able to look at what the outcome of those patients was on the trial when they were treated either with Poteligeo or the control, which was a standard of care drug. And the one thing that we saw was that the response of those patients and the safety to the two drugs was very similar. In other words, this is good news, right? Because number one, we have information on how well tolerated this particular drug is in the African American population. That’s something they had for a lot of trials that was not available. So, that was a big a big important finding. But the other one is that when patients are treated on a clinical trial where African Americans have all the attention—early intervention, careful monitoring in access to new investigational drugs—their outcomes can be just as good as anybody else. This also was shown by a study that we did from the Maverick, another study from Emory University, Dr. Allen is doing a very large study in collaboration with several different centers, including Thomas Jefferson, looking at survival across different academic institutions in black patients with CTCL. And if we look at the survivals, there’s no disparity in outcome once patients are treated in centers that know how to treat patients with CTCL. I think that’s a very important message to get out.
Daniel Levine: Pierluigi Porcu, co-leader of the Immune Cell Regulation and Targeting Program at the Sydney Kimmel Cancer Center at Jefferson Health, and Lauren Walrath, vice president of Public Affairs for Kyowa Kirin. Pierluigi, Lauren, thanks so much for your time today.
Lauren Walrath: Thank you.
Pierluigi (Pigi) Porcu: Thank you. Was a pleasure .

This transcript has been edited for clarity and readability