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Chapter 65: Cutaneous T-Cell Lymphoma

DEFINITION

  • Mycosis fungoides and its variant Sézary syndrome, the two principal types of cutaneous T-cell lymphoma (CTCL), are malignant proliferations of mature memory T lymphocytes of the phenotype CD4+CD45RO+ (memory T cells), which invariably involve the skin.

  • Other types of lymphoma may also have prominent skin involvement (see Table 65–1).

  • In cases of CTCL in which erythrodermia is a prominent feature, the major considerations are shown in Table 65–2.

TABLE 65–1WORLD HEALTH ORGANIZATION–EUROPEAN ORGANIZATION FOR RESEARCH AND TREATMENT OF CANCER CLASSIFICATION OF PRIMARY CUTANEOUS T-CELL AND NATURAL KILLER CELL LYMPHOMAS
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TABLE 65–1 WORLD HEALTH ORGANIZATION–EUROPEAN ORGANIZATION FOR RESEARCH AND TREATMENT OF CANCER CLASSIFICATION OF PRIMARY CUTANEOUS T-CELL AND NATURAL KILLER CELL LYMPHOMAS

  1. Mycosis Fungoides (MF)

    1. MF variants and subtypes

      1. Folliculotropic MF

      2. Pagetoid reticulosis

      3. Granulomatous slack skin

  2. Sézary Syndrome

  3. Adult T-Cell Leukemia/Lymphoma

  4. Primary Cutaneous CD30+ Lymphoproliferative Disorders

    1. Primary cutaneous anaplastic large cell lymphoma

    2. Lymphomatoid papulosis

  5. Subcutaneous Panniculitis-Like T-Cell Lymphoma

  6. Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type

  7. Primary Cutaneous Peripheral T-Cell Lymphoma, Unspecified

    1. Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma (provisional)

    2. Cutaneous γδ T-cell lymphoma (provisional)

    3. Primary cutaneous CD4+ small-/medium-size pleomorphic T-cell lymphoma (provisional)

  8. Precursor Hematologic Neoplasm

    1. CD4+/CD56+hematodermic neoplasm (blastic NK-cell lymphoma)

Source: Williams Hematology, 9th ed, Chap. 103, Table 103–1.

TABLE 65–2CLASSIFICATION OF ERYTHRODERMIC CUTANEOUS T-CELL LYMPHOMA
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TABLE 65–2 CLASSIFICATION OF ERYTHRODERMIC CUTANEOUS T-CELL LYMPHOMA
Erythrodermic Subset (T4) Preexisting MF Blood
Sézary syndrome Rarely Leukemia: B2

Erythrodermic mycosis

Fungoides

 Always Normal or minimally abnormal: B0–B1
Erythrodermic cutaneous T-cell lymphoma, not otherwise specified Absent Normal or minimally abnormal: B0–B1
MF, mycosis fungoides.

Source: Williams Hematology, 9th ed, Chap. 103, Table 103–4.

EPIDEMIOLOGY

  • CTCL is more common in males than females.

  • Median age at diagnosis is 55 years.

  • In the United States, there are approximately 3000 cases per year, representing about 3% of lymphomas.

  • Americans of African descent have a higher incidence and more progressive disease than Americans of European descent.

  • Asians and Hispanics are much less often affected.

  • The etiology is unknown.

CLINICAL FINDINGS

  • Patients usually present with nonspecific skin lesions (chronic dermatitis) occurring years before diagnosis.

  • Early in disease, patients are often diagnosed with eczema (spongiotic dermatitis), psoriatic-like dermatitis, or other nonspecific dermatoses associated with pruritus.

  • Histologic diagnosis may be difficult in early stages. Neoplastic infiltrates may be minimal, masked by normal inflammatory cells, and the neoplastic mature CD4+ phenotype may be misinterpreted as normal inflammatory cells.

  • Mycosis fungoides may be divided into patch stage (patch-only disease), plaque stage (both patches and plaques) and tumor stage (more than one tumor along with patches and plaques).

  • A patch is defined as a flat lesion with varying degrees of erythema with fine scaling; a plaque is defined as a demarcated, erythematous, brownish lesion, with variable scaling of at least 1 ...

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