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Lymphoma - Non B cell neoplasms

T/NK cell disorders

Primary cutaneous gamma-delta T-cell lymphoma


Reviewer: Dragos Luca, M.D. (see Reviewers page)
Revised: 26 October 2011, last major update August 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

Definition
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● Primary cutaneous γδ T-cell lymphoma (PCGD-TCL) is a lymphoma composed of a clonal proliferation of mature, activated γδ T-cells with a cytotoxic phenotype (WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, 4th Edition, Lyon 2008)

Terminology
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● Includes γδ subcutaneous panniculitis-like T-cell lymphoma
● May be part of muco-cutaneous γδ T-cell lymphoma, a more encompassing disease

Epidemiology
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● 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
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● Usually limited to skin
● Often generalized skin lesions, preferentially affecting extremities

Etiology
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● 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
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● 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

Case reports
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● Fatal case (Am J Dermatopathol 2008;6:593)
● 40 year old man with Behcet disease (Pathologica 2008;100:166)
● Associated with hemophagocytic syndrome (Am J Dermatopathol 1994;16:426)
● During treatment with etanercept (TNF-α antagonist) for rheumatoid arthritis (Acta Derm Venereol 2009;89:653)

Treatment and prognosis
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● 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)

Postulated normal counterpart
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● Functionally mature and activated cytotoxic γδ T-cells of the innate immune system

Gross description
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● Multiple plaques and tumors resembling disseminated pagetoid reticulosis

Gross images
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Various images

Micro description
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● 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
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Various images

Positive stains
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● CD3, CD56, TIA1, granzyme B, perforin, variable CD2 and CD7, TCRδ

Negative stains
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● CD4 and CD8 (usually), CD30, CD57, βF1, TdT, EBV

Genetics and Molecular
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● 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 images
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PCR images

Additional references
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Arch Dermatol 2000;136:1024, Am J Surg Pathol 2004;28:719

End of Lymphoma - Non B cell neoplasms > T/NK cell disorders > Primary cutaneous gamma-delta T-cell lymphoma


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