The Role of the Dermatologist in Diagnosing and Caring for CTCL Patients (Chapter 2)
Larisa Geskin, MD, Professor of Dermatology at Columbia University Medical Center and Director of the Comprehensive Skin Cancer Center at the Division of Cutaneous Oncology in the Department of Dermatology, discusses the role of dermatologists in diagnosing and caring for cutaneous T-cell lymphoma (CTCL) patients.
The diagnosis of CTCL is often challenging; as a result, delays in diagnosis (and subsequently work-up and treatment) can be significant. Part of the reason is the variability in how individual patients present with CTCL and its subtypes. Because mycosis fungoides progresses slowly, some patients may not experience progression beyond their initial symptoms, even beyond 10 years. Patients with mycosis fungoides or Sézary syndrome also have overlap in manifestations; in fact, Sézary syndrome was once classified as a malignant, leukemic variant of mycosis fungoides but is now recognized as a distinct CTCL subtype.
Diagnosis is usually made with a patient history, complete physical exam, blood tests, biopsy of skin lesions, computed tomography imaging, and sometimes lymph node biopsy and/or bone marrow biopsy. These methods can also be useful in determining the stage of disease, especially whether the lymph nodes have been involved and whether the cancerous cells have spread to blood and other organs. In addition to eczema and psoriasis, the differential diagnosis may include nonspecific dermatitis, lichen, lupus, pseudolymphoma, parapsoriasis, and toxidermia.
In early-stage CTCL, patients most often visit dermatologists for the diagnosis of their skin lesions, and typically manage it using skin-directed treatment. However, as CTCL progresses, oncologists play an important, collaborative role. The oncology team will commonly manage CTCL with systemic, targeted treatments, radiation approaches, sometimes chemotherapy for patients with Sézary syndrome, advanced disease, refractory CTCL, and other aggressive forms. Clinical trials may also be considered.
Some of the more recent directions in treatment have focused on targeted systemic therapies or targeted monoclonal antibody treatment (e.g., CCR4 or CD30). For example, brentuximab vedotin is approved for CD30-positive mycosis fungoides. Mogamulizumab, in contrast has been approved for certain MF and SS and targets specific receptors on the malignant cells (i.e. CCR4).
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