[531] Five Cases of Indolent Gamma Delta T-Cell Lymphoma (TCL) Localized to the Subcutaneous Panniculus.

Xuan Wang, Cynthia M Magro. New York Presbyterian Hospital-Weill Cornell Medical Center, NY

Background: The WHO EORTC classification of lymphoma designates subcutaneous panniculitis-like T-cell lymphoma (SPTCL) exclusively to alpha beta (αβ) T-cell phenotype. Those of gamma delta (γδ) T-cell type are considered as other forms of primary cutaneous γδ TCL. The basis for this classification was the more indolent course of αβ form compared with the poor 5-year survival of γδ form. However, rare case reports exist of a waxing and waning clinical course in γδ form of SPTCL.
Design: Skin biopsies were studied from 5 patients with γδ TCL localized to the subcutaneous panniculus. A retrospective analysis of clinicopathologic features and follow-up was done.
Results: Clinical features of the 5 γδ TCLs are tabulated below.

Table 1
caseage at onsetsexlocationfollowup periodtreatmentoutcome
125Fleg7yfludarabinecomplete remission for 5y
233FNA7yCHOP + allo stem cell transplantremission
336Farm thigh leg7ysystemic chemo 5y after onsetdied 7y after onset
476Faxilla0.5ysurgical resection with local radiationremission
545Fwrist leg chest8yfludarabin 6y after onsetdied 8y after onset
y: years

Histologic features, IHC results of selected T-cell markers and cytotoxic proteins are shown too.

Table 2
casedermal extension and adnexal involvementadipocyte rimmingCD5CD7CD4/CD8TIA-1/granzymeCD56/CD62L
1++---/-++/NA
2++-+-/-+-/-
3++-+-/-++/-
4++-+-/-++/NA
5++-+-/-++/-


Case 3 was originally diagnosed as lupus profundus and treated with Plaquenil until 5y after onset. Earlier biopsies were better differentiated without the degree of hemophagocytosis or macrophage infiltration, although retrospective IHC studies revealed an identical phenotypic profile. Case 5 was originally interpreted as panniculitis and treated with antibiotics and steroids until 6y after onset. These 2 patients died 7 and 8 years after original presentation, due to complication of lymphoma and immunosuppression, respectively. Patient 5 was lymphoma-free at the time of death.
Conclusions: We present a series of 5 patients with γδ TCLs localized to the subcutis whereby the clinical course was indolent. Four were younger women with lesions in extremities, all of whom were alive 5y after presentation. All responded to chemotherapy. Complete remission was achieved in 4 cases. The exact pathogenetic mechanism that determines the outcomes in SPTCL is unclear. In this regard, the prior classification which grouped all primary subcutaneous T-cell lymphomas under SPTCL seems more reasonable.
Category: Dermatopathology

Wednesday, March 2, 2011 1:00 PM

Poster Session VI # 111, Wednesday Afternoon

 

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