Anne Wood Beaven, MD, Associate Professor of Medicine and Vice Chief of Operations, Division of Hematology at University of North Carolina School of Medicine, gives an overview of cutaneous T-cell lymphoma (CTCL).

As Dr. Beaven explains, CTCL belongs to the non-Hodgkin lymphoma family as a rare group of malignancies. CTCL attacks the immune system, specifically, the lymphatic system which affects B-cells and T-cells. Compared to other T-cell lymphomas, CTCL involves malignant T-cells migrating to, and collecting in, cutaneous tissue. This makes diagnosis challenging as the initial signs are skin-related and, therefore, overlap with many other dermatologic disorders. Additionally, CTCL variants present overlapping symptomatology, making it difficult to diagnose between CTCL subtypes. Hence, histopathologic features must be correlated with the clinical presentation to confirm diagnosis.

Many forms of CTCL are relatively indolent compared with other T-cell lymphomas, but there are aggressive subtypes. This is illustrated by the two most common forms of CTCL: mycosis fungoides and Sézary syndrome. Although mycosis fungoides is considered a slow-growing variant, Sézary syndrome is aggressive and generally has a poor prognosis. Importantly, even the indolent subtypes can progress in some patients and become difficult to manage.

For more information about CTCL and other rare cancers, visit checkrare.com/diseases/cancers/

November 2019 - Dr. Joi Carter provides an overview of what cutaneous lymphomas are and how they are diagnosed and staged.

Event:
Patient Educational Forum in Framingham, MA.

Clinician:
Joi Carter, MD, Dartmouth-Hitchcock Medical Center, Lebanon

Cutaneous T-Cell Lymphoma: Symptoms, Treatment, and Diagnosis
A conversation with Dr. Kimberly Bohjanen an MyHealthTeams

Larisa Geskin, MD of Columbia University Medical Center, New York, NY gives an overview of her talk on chemotherapy for cutaneous T-cell lymphoma (CTCL) given as part of a series of lectures on the role of chemotherapy in CTCL, melanoma and squamous cell carcinoma held at the 2016 World Congress on Cancers of the Skin (WCCS) and the Congress of the European Association of Dermato-Oncology (EADO) in Vienna, Austria. Dr Geskin explains that chemotherapy may play little role in the management of these diseases, particularly in the case of CTCL. Multi-agent chemotherapy may only play a role in the palliative setting in CTCL, which is related to the fact that CTCL is a chronic and incurable disease. Therefore, using a potent immunotherapy approach is better than using toxic chemotherapy. For a subset of patients, mostly end stage patients who do not have other options or patients who are in severe pain, multi-agent chemotherapy can be used.

Auris Huen, MD, PharmD, Assistant Professor, MD Anderson Cancer Center, Department of Dermatology provides an overview of cutaneous T-cell lymphoma (CTCL).

Cutaneous T-cell lymphoma belongs to the non-Hodgkin lymphoma class of hematologic T-cell lymphoproliferative disorders. Cutaneous T-cell lymphoma is a rare group of malignancies, with an incidence of 6.4 cases per 1 million people. This form of T-cell lymphoma represents around 70% of primary cutaneous lymphomas.

Cutaneous T-cell lymphoma attacks the the body’s immune system, specifically, the lymphatic system, affecting the two types of white blood cells (lymphocytes): B-cells and T-cells. Whereas the B-lymphocytes act to neutralize the pathogens, the main job of the T-lymphocytes is to attach to these foreign cells, viruses, or cancerous growths, and directly destroy them.

Compared with other T-cell lymphomas, a distinguishing feature of CTCL is implied by the name: malignant T-cells migrating to, and collecting in, cutaneous tissue. Diagnosis can be challenging, because the initial signs and symptoms are largely skin-related and overlap with those of many other dermatologic disorders. Adding to the challenge, CTCL variants present with overlapping symptomatology, and correct identification of the CTCL subtype is key to both treatment and prognosis. Histopathologic features must be correlated with the clinical presentation to confirm the diagnosis.

Many forms of CTCL are relatively indolent compared with other T-cell lymphomas, but there are aggressive subtypes. This is illustrated by the two most common forms of CTCL: mycosis fungoides and Sézary syndrome. Although mycosis fungoides is considered a slow-growing variant, Sézary syndrome is aggressive and generally has a poor prognosis. Importantly, even the indolent subtypes can progress in some patients and become difficult to manage.

Michi Shinohara, MD

Associate Professor; Division of Dermatopathology
Department of Laboratory Medicine & Pathology; University of Washington

Cutaneous T-cell lymphomas (CTCLs) are non-Hodgkin lymphomas with protean clinical manifestations the primarily occur in the skin. Mycosis fungoides (MF), primary cutaneous CD30 positive lymphoproliferative disorders, and Sézary syndrome (SS) are the most common subtypes of CTCL, with MF comprising the majority (less than 60%) of cases. MF is a neoplasm of mature, skin-homing effector memory T-helper (TH) cells. Diagnosis of early MF is notoriously challenging because of significant clinical and histologic overlap with benign skin conditions. We will discuss some of the reasons for this difficulty in diagnosing MF, the role of molecular studies in diagnosis and risk stratifying MF, and some newer data on the genetic landscape of MF with a glimpse of the potential for precision medicine in the treatment of MF.