Navigating Diagnostic and Therapeutic Challenges in Primary Cutaneous Gamma/Delta T-Cell Lymphoma: A Case Study of Fatal Outcomes Within Two Months

被引:0
作者
Shaker, Nada [1 ]
Blankenship, Heath [2 ]
Masatkar, Vaishali [2 ]
Niu, Shuo [3 ]
Sangueza, Omar P. [2 ]
机构
[1] Ohio State Univ, James Canc Hosp, Wexner Med Ctr, Dept Pathol, 410 W 10th Ave, Columbus, OH 43210 USA
[2] Wake Forest Univ, Sch Med, Dept Pathol & Dermatol, Med Ctr Blvd, Winston Salem, NC USA
[3] Wake Forest Univ, Sch Med, Dept Pathol, Sect Comparat Med, Med Ctr Blvd, Winston Salem, NC USA
关键词
primary cutaneous gamma/delta T-cell lymphoma; primary cutaneous T-cell lymphoma with gamma-delta phenotype; primary cutaneous T-cell lymphoma; primary cutaneous lymphoma; T-cell lymphoma; GAMMA-DELTA; MYCOSIS-FUNGOIDES; EXPRESSION; PHENOTYPE; PROGNOSIS;
D O I
10.1097/DAD.0000000000002743
中图分类号
R75 [皮肤病学与性病学];
学科分类号
100206 ;
摘要
Primary cutaneous gamma/delta T-cell lymphoma (PCGD-TCL) is a rare yet highly aggressive subtype of primary cutaneous lymphoma. Characterized by its challenging diagnosis and poor prognosis, PCGD-TCL presents unique clinical and histopathological features that distinguish it from other primary cutaneous lymphoma subtypes. Here, we report the case of a 75-year-old man who initially presented with multiple erythematous indurated plaques over his back and bilateral lower extremities. The initial biopsy suggested primary cutaneous T-cell lymphoma (PCTCL) with a CD30-negative phenotype. However, within a 2-month interval, the disease progressed rapidly, manifesting as extensive skin involvement across the chest and upper extremities. A repeat skin biopsy was performed, revealing dermal atypical lymphocytes without epidermotropism. Immunohistochemical analysis demonstrated positivity for CD3, CD5, and CD4, as well as T-cell receptor delta (TCR delta) expression, along with the loss of CD8 and CD30 expression. These findings were consistent with a diagnosis of PCGD-TCL. Despite therapeutic interventions, including systemic treatments, the patient's condition deteriorated rapidly, ultimately leading to his demise within a month of receiving the PCGD-TCL diagnosis. This case highlights the diagnostic complexities associated with PCGD-TCL, emphasizing the importance of careful histopathological examination and immunophenotypic characterization. Given its aggressive nature and propensity for rapid dissemination, early recognition of PCGD-TCL is paramount for initiating appropriate therapeutic interventions. However, effective treatment options for PCGD-TCL remain limited, and the disease typically carries an unfavorable prognosis. Further research is needed to elucidate the underlying molecular mechanisms driving the pathogenesis of PCGD-TCL, to identify novel therapeutic targets, and to improve patient outcomes. In addition, increased awareness among clinicians and pathologists regarding the clinical presentation and diagnostic criteria of PCGD-TCL is crucial for facilitating timely diagnosis and management of this challenging malignancy.
引用
收藏
页码:593 / 596
页数:4
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