Primary Cutaneous CD4+ Small/Medium Pleomorphic T-cell Lymphoma

Primary Cutaneous CD4+ Small/Medium Pleomorphic T-cell Lymphoma

Aaron Auerbach, MD, PhD

Low-power view of a primary cutaneous CD4(+) small/medium pleomorphic T-cell lymphoma shows a dense lymphoid infiltrate filling the dermis in a diffuse pattern of infiltration.

At higher power magnification, the lymphocytes are small to medium in size and show moderate atypia and pleomorphism image.



  • Primary cutaneous CD4(+) small/medium pleomorphic T-cell lymphoma (SMPTCL)


  • Idiopathic T-cell lymphomatoid hyperplasia


  • T-cell lymphoma of skin with small to medium-sized CD4(+) T cells, and usually indolent clinical course

    • Without typical patches/plaques of mycosis fungoides

  • Provisional entity in 2008 WHO Classification of Tumors of Hematopoietic and Lymphoid Tissue

    • Nonaggressive natural history, but worrisome histologic and molecular features



  • Cause of this lymphoma unknown Postulated Normal Cell Counterpart

  • CD4(+) helper T cell



  • Incidence

    • Rare

      • 2% of cutaneous T-cell lymphomas in the Dutch and Austrian Cutaneous Lymphoma Group Registry

  • Age

    • Median: 53 years; range: 3-90 years

  • Gender

    • M:F = 0.5:1


  • Head and neck area most common

    • Upper torso often

    • Lower extremities rare


  • Single plaque or tumor

    • Less commonly > 1 tumor nodule

    • Less commonly ≥ 1 papules

    • No lesions (patches/plaques) typical of mycosis fungoides

  • Usually asymptomatic other than skin lesions

    • No lymphadenopathy

    • No systemic disease

Laboratory Tests

  • No known laboratory abnormalities

Natural History

  • Does not usually disseminate

    • Rarely, local recurrence is seen


  • Excision

  • Radiation therapy

  • Usually nonaggressive therapy

    • Especially if single lesion


  • Mostly favorable outcome, especially if solitary lesion or localized to skin

    • Better prognosis than secondary cutaneous T-cell lymphomas

    • Better prognosis than primary cutaneous peripheral T-cell lymphoma, not otherwise specified

  • Nonaggressive disease

    • Overall 5-year survival: 60-80%

    • Disease-specific 5-year survival up to 75%

  • Good prognostic indicators

    • Solitary lesion

    • Disease localized to skin

  • Worse prognostic indicators

    • Multiple lesions

    • Large lesions


General Features

  • Usually single, less often multiple firm lesion(s)


Histologic Features

  • Dense dermal infiltrate

    • Sometimes involves subcutaneous tissue

    • Usually nonepidermotropic

      • Rarely focal epidermotropism

  • Growth pattern

    • Diffuse

    • Nodular

  • Size of T cells

    • Small or medium in most cases

    • Large cells can also be seen

      • When present, < 30% of total cells

  • Pleomorphic with some nuclear irregularity

  • Other features

    • Background reactive infiltrate is fairly common

      • Small reactive lymphocytes, eosinophils, and histiocytes

    • ↑ reactive-appearing B cells also often present

    • Granulomatous inflammation can be seen

Cytologic Features

  • Rarely diagnosed by cytology



  • T-cell antigens positive

    • CD2(+), CD3(+), CD5(+), CD7(+)

    • Rarely loss of 1 or more T-cell antigens

      • CD7 > CD5, CD2

    • CD4(+), CD8(−)

  • Expresses follicular helper T-cell markers

    • PD1(+), CXCL13(+), Bcl-6(+)

  • Cytotoxic markers negative

    • TIA, GZM-B, and perforin

  • CD30(−), LMP1(−)


  • No known specific abnormalities

In Situ Hybridization

  • Epstein-Barr virus small-encoded RNA (EBER) negative


  • TCR gene rearrangement clonal

    • 60% in one series

  • IgH immunoglobulin gene rearrangements polyclonal

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Primary Cutaneous CD4+ Small/Medium Pleomorphic T-cell Lymphoma
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