An Overview of Cutaneous T-Cell Lymphoma (CTCL)
Stefan M. Schieke, MD, Assistant Professor of Dermatology at University of Wisconsin-Madison, 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.
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.
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