Dr. Jo-Ann Latkowski discusses the dermatologist’s role in diagnosis and treatment of less common CD30 lymphoproliferative disorders, including a tried-and-true approach to counseling patients.
Jo-Ann Latkowski, MD, is Associate Professor of Dermatology, NYU School of Medicine, New York, New York.
“Our most common type of cutaneous lymphoma is mycosis fungoides [but] there are many other types of skin lymphomas we see in our own offices, maybe not on a daily basis, but that really needed to be highlighted,” said Jo-Ann Latkowski, MD, who presented “Managing Patients With Non-MF Cutaneous Lymphoma” at the 2022 AAD Annual Meeting.
“I wanted to reassure the audience of dermatologists that they could diagnose these non-MF cutaneous lymphomas, stage them, and treat them in their own offices and this would be something where, most of the time, a hematologist oncologist wouldn’t need to be involved.”
The two most common CD30 lymphoproliferative disorders are lymphomatoid papulosis and anaplastic large cell lymphoma, said Dr. Latkowski.
“…the CD30 marker on lymphoid cells that you see in the skin biopsy does not automatically mean that you have this type of lymphoproliferative disease because you could see it in other cell types.”
Those include both benign and malignant, said Dr. Latkowski.
“But when you do see it in a large number of T cells, especially in the very specific clinical findings of the CD30 lymphoproliferative disease, it does take you away from the most common type of skin lymphoma, mycosis fungoides, which in most cases is CD30 negative, to a less common CD30 lymphoproliferative disorder.”
According to Dr. Latkowski, special attention is warranted when it comes to lymphomatoid papulosis, not only because it is the most common type of CD30 process encountered in the dermatology office, but because it also presents the most difficulty when counseling patients.
“They’re erythematous papules that come up in crops. They could necrose or get a scab on it, and by definition, they go away on their own. And if you ever see it in your own office, you would do a punch biopsy to make a diagnosis.”
It’s Dr. Google that really makes patient counseling tricky, said Dr. Latkowski.
“They’re going to Google it and it’s going to say, ‘you have a cutaneous lymphoma,’ or cancer. But you need to tell the patient that the diagnosis of lymphomatoid papulosis has a five-year survival of 100%. Nobody dies of lymphomatoid papulosis, but it is in the [World Health Organization–European Organization for Research and Treatment of Cancer] WHO-EORTC cutaneous lymphoma classification as a cancer because of its high association with other skin cancers like mycosis fungoides and anaplastic large cell lymphoma.”
Specifically, according to the National Cancer Institute, lymphoproliferative disorder is a “disease in which cells of the lymphatic system grow excessively. Lymphoproliferative disorders are often treated like cancer.”
Keeping a Close Watch
To monitor patients, clinical follow up for lymphomatoid papulosis (LyP) should happen twice a year, said Dr. Latkowski.
“I tell my patients, you’ll get to know me well, we’ll meet every six months.”
One of the things patients need to know is that frequency of lymphomatoid papulosis papules varies.
“Some patients get 10 to 20 at a time, others get 10 a year—they’re not so active.
In between follow ups, it’s the patient’s job to monitor papules for two red flag scenarios, said Dr. Latkowski.
“If they get a small papule, like a pimple-like lesion that doesn’t go away in 8 weeks, 10 weeks, or if it grows larger in size, like a tumor size, then you as the dermatologist need to be informed because that could mean this LyP has now become anaplastic large cell lymphoma, which again, has an excellent prognosis.”
The other scenario includes warning signs of mycosis fungoides, she said.
“The other time I have my patients with LyP contact me or come in sooner is [if] they notice dry patches or eczema-like eruption in the non-sun-exposed areas—on the breast, the buttock, inner thighs, inner arms—and that would make me suspicious for mycosis fungoides, the other skin lymphoma that LyP is highly associated with.”
The Oncologist Referral
Although lymphomatoid papulosis and early-stage mycosis fungoides have an excellent non-life-threatening prognosis, some patients may still want to see an oncologist, said Dr. Latkowski.
“I don’t blame those patients at all. [From their perspective], ‘You’re only a dermatologist. I have a cancer I want to see a cancer doctor.’”
However, you don’t want them to see someone to be over staged, over treated, and potentially not provide the message that they have a good to excellent prognosis, said Dr. Latkowski.
“A lot of my patients will go to an oncologist. They get over staged…. They get bone marrows, they get pet CTs for lymphomatoid papulosis, sometimes they get over treated with radiation for lymphomatoid papulosis, and again, by definition, lymphomatoid papulosis goes away on its own, so you don’t really need to treat it.”
According to Dr. Latkowski, the key is to be proactive with these patients.
“I will steer them to an oncologist who treats skin lymphoma, who understands the difference between systemic lymphoma and skin lymphoma.”
All of this strategic counseling for LyP took years to cultivate, said Dr. Latkowski.
“These days patients have Googled it way before they come to see you and so they have their own idea of what it is, and it’s really hard to explain that they have something that’s going to come and go. It’s considered a cancer, but it’s considered a cancer because it’s significantly associated with other skin lymphomas.”