Lymphoma, Cutaneous T-Cell

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ICD Codes

202.10 – Mycosis fungoides
202.20 – Sezary's disease unspecified site

C84.00 – Mycosis fungoides, unspecified site


Primary cutaneous lymphomas can be divided into T-cell and B-cell lymphomas that involve the skin and various extracutaneous sites. Cutaneous T-cell lymphomas (CTCLs) account for 75%-80% of these lymphomas and are a heterogeneous group of neoplasms that vary considerably in their clinical presentation, histology, immunophenotype, genetic landscape, and prognosis. It is seen most often in the elderly, but it can occur in patients of all ages. The most recent classification scheme delineates the frequency and survival of patients with the varying types of cutaneous lymphomas and is outlined in Management Pearls section. For physicians not specialized in treating CTCL, practical guidelines exist with respect to the diagnosis, classification, and staging of CTCL. They are outlined in Diagnostic Pearls and Management Pearls. Most importantly, a clear diagnosis of CTCL must be made and may require large or multiple biopsies as well as specialized testing of the biopsy specimens. Typing of the CTCL and staging are important to determine the extent of disease and treatment strategy.

Mycosis fungoides and its variants, Sézary syndrome, lymphomatoid papulosis, and cutaneous anaplastic large cell lymphoma, make up 90% of all CTCL cases and will be discussed in this review. Note that for clinicians seeking additional information, CTCL has been the subject of several recent in-depth reviews.

Mycosis Fungoides
Mycosis fungoides (MF) is the most common type of CTCL, accounting for 50% of all primary CTCL cases. Erythematous patches and plaques with fine scale and tumors that anatomically favor the buttocks and sun-protected areas of the trunk and limbs characterize this subtype. The etiology remains unclear. Current hypotheses propose that persistent antigenic stimulation occurs and that CD8+ T-cells play a critical role. MF takes on an indolent course over years to decades. Associated features include pruritus, poikiloderma, and ulceration. Extracutaneous involvement correlates with generalized lesions and erythroderma. Neoplastic T-cells demonstrate a memory T-cell phenotype: CD3+, CD4+, CD45RO+. MF staging requires CTCL physician specialists.

Sézary Syndrome
Sézary syndrome (SS) is a rare type of CTCL accounting for less than 5% of all primary CTCL cases. Erythroderma, generalized lymphadenopathy, and neoplastic T-cells (Sézary cells) in the skin and peripheral lymph nodes classically characterize SS. Associated features include severe pruritus, palmoplantar hyperkeratosis, lichenification, edema, and exfoliation. The etiology remains largely unknown. Immunophenotyping demonstrating a CD4:CD8 ratio of >10, an absolute count of >1,000 Sézary cells per µL, and demonstration of T-cell clonality in the peripheral blood are diagnostic criteria.

Lymphomatoid Papulosis
Lymphomatoid papulosis (LyP) accounts for 15% of all primary CTCL cases and is characterized by CD30+ lymphoproliferation on histology and red to brown papules and nodules that take on a chronic, intermittently recurring, clinical course. It is unclear why some individuals experience spontaneous resolution and others have tumor progression. The etiology remains largely unknown. The papules and nodules may demonstrate crust, necrosis, and hemorrhage. LyP lesions are in varying stages of healing and can spontaneously resolve within 2 months of time. The cutaneous eruption may vary in number from few to more than 100. Lesions favor the trunk and extremities, are asymptomatic, and can vary in duration from months to decades. The prognosis is excellent.

See also Lymphomatoid Papulosis in VisualDx.

Primary Cutaneous Anaplastic Large Cell Lymphoma
Primary cutaneous anaplastic large cell lymphoma (C-ALCL) accounts for 10% of all primary CTCL cases and is characterized by CD30+ lymphoproliferation on histology and solitary nodules or tumors that ulcerate on clinical exam. Microscopically, these cells have a characteristic morphology with anaplastic large cells with round or irregularly shaped nuclei. Similar to LyP, this condition demonstrates a chronic, intermittently recurring, clinical course. Approximately 10% of individuals will experience extracutaneous involvement. However, the prognosis remains excellent.

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