A fatal case of primary cutaneous gamma–delta T‐cell lymphoma complicated by HLH and cardiac amyloidosis

Gibson, Juliet Fraser; Kapur, Lucy Harn; Sokhn, Joseph; Xu, Mina L.; Foss, Francine M.

doi:10.1002/ccr3.142

Cited by 8 publications

(2 citation statements)

References 23 publications

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“…It was previously regarded as a variant of subcutaneous, panniculitis-like T-cell lymphoma (SPTCL), but was separated from this group because of significant clinical and phenotypic differences [ 2 ]. PCGD-TCL represents less than 1% of all lymphomas [ 1 , 3 , 4 , 5 ].…”

Section: Introductionmentioning

confidence: 99%

A Previously Unrecognized Granulomatous Variant of Gamma-Delta T-Cell Lymphoma

et al. 2021

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Primary cutaneous γδ T-cell lymphoma (PCGD-TCL) is an extremely rare and aggressive T-cell neoplasm with complex heterogeneity. We present a series of two patients who presented with firm, subcutaneous nodules and were diagnosed with PCGD-TCL. In both cases, biopsies demonstrated a both superficial and deep adnexotropic infiltrate comprised of angiocentric, medium- to large-sized atypical lymphocytes. The infiltrate extended into the panniculus. Immuno–histochemical stains highlighted atypical lymphocytes that expressed CD3, CD8 and CD56 but were negative for EBV ISH. A brisk histiocytic response with focal aggregation into granulomas was highlighted with a PG-M1 stain. The atypical lymphocytes were positive for gene rearrangements on a TCR delta stain and negative for βF-1. CT and PET scan in one of the two patients demonstrated diffuse, subcutaneous, ground-glass foci; hypermetabolic soft tissue nodules; and lymphadenopathy in the lungs, as well as splenomegaly. A diagnosis of histiocyte-rich PCGD-TCL was rendered. A histiocyte-rich, granulomatous variant of γδ T-cell lymphoma is extremely rare. Its potentially misleading resemblance to inflammatory granulomatous conditions could pose a diagnostic pitfall in this already challenging condition. This variant may resemble granulomatous mycosis fungoides and granulomatous slack skin syndrome, but it has a distinct, aggressive clinical outcome.

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Section: Introductionmentioning

confidence: 99%

A Previously Unrecognized Granulomatous Variant of Gamma-Delta T-Cell Lymphoma

et al. 2021

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“…PCGDTCL is an extremely rare malignancy amounting to <1% of all skin lymphomas. Accordingly, only a small number of cases have been reported making PCGDTCL a poorly understood lymphoma (3)(4)(5)(6). In those cases reported, the course of PCGDTCL was mostly short and highly aggressive with resistance to chemo-and radiotherapy (7,8).…”

Section: Introductionmentioning

confidence: 99%