T-cell lymphomas: From diagnosis to treatment and beyond

T-cell lymphomas can be challenging to identify and manage. To discuss the diagnostic and treatment journey for patients with these cancers, hematologist-oncologist Colin Thomas, MD, and dermatopathologist Jina Chung, MD, recently joined the Penn Medicine Physician Interviews podcast. Dr. Thomas is an Assistant Professor of Clinical Medicine (Hematology-Oncology) and Dr. Chung is an Assistant Professor of Clinical Dermatology at Penn Medicine.

Representing 10 percent to 15 percent of all non-Hodgkin lymphomas, the T-cell lymphomas have many subtypes, and manifest in various parts of the body, including the lymph nodes, blood, viscera, and skin.

“Because it’s a very heterogeneous group of cancers with many different subtypes, treatments and prognosis really hinge on knowing exactly what the specific subtype is,” Dr. Thomas says. “It really is a journey from diagnosis to treatment to get the disease under control—and having the right diagnosis is the map in that journey.”

Cutaneous T-cell lymphomas

T-cell lymphomas often arise in the skin, initially presenting as a rash that may be challenging to identify. “It can take an average of four to five biopsies and sometimes four to five years to get to the correct diagnosis,” Dr. Chung says.

At Penn Medicine, dermatopathologists specialized in lymphoma play key roles in making a diagnosis and identifying the subtype with immunophenotyping and, in some cases, genetic testing. In early stages, dermatologists take the lead on treatment , drawing on tools such as topical therapies, phototherapy, and oral or injectable medications.

In more advanced stages, and for aggressive subtypes, hematologist-oncologists typically manage frontline therapies. These may include chemotherapy, targeted therapies, and immunotherapies. “It’s such a complex treatment ladder,” Dr. Chung says, requiring close collaboration between dermatology and oncology. “I can't stress enough that this is a multidisciplinary team approach.”

T-cell lymphoma research and treatment

The diagnosis and treatment of non-cutaneous (nodal) T-cell lymphomas can also be quite complex. Frontline therapies for nodal T-cell lymphomas often involve standard chemotherapy cocktails, but a variety of other novel therapies may also play an important role. “There’s certainly a toolbox of systemic therapies for patients,” Dr. Thomas says.

However, in many cases—such as relapsed/refractory nodal T-cell lymphomas, an allogeneic bone marrow transplant is the only option for achieving long-term remission. That means earlier treatment decisions can be important as a bridge to transplant, Dr. Thomas adds.

Penn Medicine physician-scientists are also actively involved in research to develop new therapies for T-cell lymphomas, including an ongoing clinical trial evaluating a new type of CAR T cell therapy for T-cell lymphomas — one that uses a CRISPR knockout gene editing technique to prevent T cell fratricide in CAR T cells, and does not cause T cell aplasia.

Preliminary data has been promising, Dr. Thomas says, noting a 100% overall response rate and 100% complete remission rate among the three patients studied.

Dr. Thomas observes that while the therapy is very interesting, more follow-up is needed, and questions remain, including whether the technology will be used as a bridge to an allogeneic transplant, or as a definitive therapy in itself.

Connecting with lymphoma specialists at Penn Medicine

Given the complexity of T-cell lymphomas, patients benefit from connecting early with oncologists and dermatopathologists who specialize in these diseases.  Stressing the urgency of second opinions, Dr. Thomas strongly encourages any new diagnosis of a T-cell lymphoma to have a consult with specialists at a tertiary care center.

At Penn Medicine, the multidisciplinary lymphoma team includes dermatologists, dermatopathologists, hematologists/oncologists, and radiation oncologists who work closely together to streamline care for patients. The team holds regular tumor boards and cutaneous lymphoma conferences to review pathology, discuss complex cases, and identify patients who are candidates for clinical trials.

The team also collaborates with local physicians who may be involved in the diagnosis and treatment process. When patients live far from Penn Medicine, they may receive some treatments closer to home.

“We try to make the journey as easy as possible when collaborating or acting as a consultant for outside institutions,” Dr. Chung adds.

Referrals and consultations

Dr. Thomas and Dr. Chung see patients at the Perelman Center for Advanced Medicine. For a provider-to-provider consultation, please call 877-937-7366, or refer a patient online.