A Previously Unrecognized Granulomatous Variant of Gamma-Delta T-Cell Lymphoma
| Autor: | David M. Menke, Tatsiana Pukhalskaya, Bruce R. Smoller, Olayemi Sokumbi |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
0301 basic medicine
Pathology medicine.medical_specialty PCGD-TCL CD3 Case Report primary cutaneous γδ T-cell lymphoma Dermatology histiocyte-rich PCGD-TCL Stain 03 medical and health sciences 0302 clinical medicine medicine Gamma delta T cell Histiocyte Atypical Lymphocyte biology business.industry Granulomatous slack skin medicine.disease Lymphoma 030104 developmental biology medicine.anatomical_structure RL1-803 030220 oncology & carcinogenesis biology.protein business CD8 |
| Zdroj: | Dermatopathology, Vol 8, Iss 27, Pp 221-228 (2021) Dermatopathology |
| ISSN: | 2296-3529 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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