Lymphoma and plasma cell neoplasms
T / NK cell disorders
Primary cutaneous gamma-delta T cell lymphoma

Author: Dragos Luca, M.D. (see Authors page)

Revised: 28 March 2017, last major update August 2011

Copyright: (c) 2001-2017, PathologyOutlines.com, Inc.

PubMed search: primary cutaneous gamma-delta [title] T cell lymphoma

Cite this page: Primary cutaneous gamma-delta T cell lymphoma . PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/lymphomanonBcutangammadelta.html. Accessed April 23rd, 2017.
Definition / General
Terminology
  • Includes γδ subcutaneous panniculitis-like T cell lymphoma
  • May be part of mucocutaneous γδ T cell lymphoma, a more encompassing disease
Epidemiology
  • Rare, 1% of all cutaneous T cell lymphomas
  • Slight female predominance in older studies (now equal frequencies), mean 60 years, range 34 - 77 years
Sites
  • Usually limited to skin
  • Often generalized skin lesions, preferentially affecting extremities
Etiology
  • Disease distribution corresponds to localization of normal γβ T cells
  • Role in host mucosal and epithelial defense, therefore impaired immunity and chronic antigen stimulation may be predisposing factors
Clinical Features
  • Predominantly epidermotropic: patches, plaques, deep dermal or subcutaneous nodules with variable epidermal necrosis and ulceration
  • Mucosal and extranodal dissemination frequent
  • Uncommon in lymph nodes, bone marrow or spleen
  • B symptoms in majority of patients
  • A hemophagocytic syndrome may occur
Prognostic Factors
Treatment and prognosis:
  • Resistant to multiagent chemotherapy and radiation
  • Median survival 15 months
  • More unfavorable prognosis with subcutaneous disease
  • One study showed 22 of 33 patients (66%) die within 5 years of diagnosis
  • TCRδ1 expression was an independent predictor of survival (Blood 2003;101:3407)
Case Reports
Postulated normal counterpart
  • Functionally mature and activated cytotoxic γδ T cells of the innate immune system
Clinical Images

Images hosted on other servers:

Various images

Gross Description
  • Multiple plaques and tumors resembling disseminated pagetoid reticulosis
Micro Description
  • Three major histologic patterns: epidermotropic, dermal, subcutaneous (often occurring simultaneously)
  • Involvement of epidermis, dermis and subcutaneous tissue with interface dermatitis, no grenz zone
  • Pleomorphic cells infiltrate adnexa with tumor cell necrosis, apoptosis, epidermal necrosis and rimming of fat cells; occasional angiodestruction and granulomas
  • Medium to large sized cells with coarsely clumped chromatin, occasionally large blastic cells with vesicular nuclei and prominent nucleoli
Micro Images

Images hosted on other servers:

Various images

Positive Stains
Negative Stains
Molecular / Cytogenetics Description
  • Clonal rearrangement of TRG@ and TRD@ genes
  • TRB@ may be rearranged or deleted, but not expressed
  • Vδ2 expression in cases with predominant subcutaneous involvement
Molecular / Cytogenetics Images

Images hosted on other servers:

PCR images