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Chapter 65: Cutaneous T-Cell Lymphoma

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    AMA Citation
    Cutaneous T-Cell Lymphoma. In: Lichtman MA, Kaushansky K, Prchal JT, Levi MM, Burns LJ, Armitage JO. Lichtman M.A., Kaushansky K, Prchal J.T., Levi M.M., Burns L.J., Armitage J.O. Eds. Marshall A. Lichtman, et al.eds. Williams Manual of Hematology, 9e New York, NY: McGraw-Hill; . http://hemonc.mhmedical.com/content.aspx?bookid=1889&sectionid=137390338. Accessed October 22, 2019.
    MLA Citation
    . "Cutaneous T-Cell Lymphoma." Williams Manual of Hematology, 9e Lichtman MA, Kaushansky K, Prchal JT, Levi MM, Burns LJ, Armitage JO. Lichtman M.A., Kaushansky K, Prchal J.T., Levi M.M., Burns L.J., Armitage J.O. Eds. Marshall A. Lichtman, et al. New York, NY: McGraw-Hill, , http://hemonc.mhmedical.com/content.aspx?bookid=1889&sectionid=137390338.

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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 ...

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