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原发性皮肤γ/δ T 细胞淋巴瘤的诊断与治疗挑战:两个月内致死性结局的病例研究

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

PubMed 2024/06/06(内容时间) Am J Dermatopathol Q4 · IF 0.8(JCR 2025)

研究概要

原发性皮肤γ/δ T细胞淋巴瘤(PCGD-TCL)是一种罕见但高度侵袭性的原发性皮肤淋巴瘤亚型。

中文摘要

原发性皮肤γ/δ T细胞淋巴瘤(PCGD-TCL)是一种罕见但高度侵袭性的原发性皮肤淋巴瘤亚型。PCGD-TCL以诊断困难、预后差为特征,具有独特的临床和组织病理学特征,可与其他原发性皮肤淋巴瘤亚型相区分。在此,我们报告一例75岁男性患者,最初表现为背部和双下肢多发红斑性硬化性斑块。初次活检提示原发性皮肤T细胞淋巴瘤(PCTCL),呈CD30阴性表型。然而,在2个月的时间内,疾病迅速进展,表现为胸部和上肢广泛的皮肤受累。再次行皮肤活检,显示真皮非典型淋巴细胞,无嗜表皮现象。免疫组化分析显示CD3、CD5和CD4阳性,以及T细胞受体δ(TCR delta)表达,同时CD8和CD30表达缺失。这些发现与PCGD-TCL的诊断一致。尽管采取了包括全身治疗在内的治疗干预,患者病情仍迅速恶化,最终在确诊PCGD-TCL后一个月内死亡。本病例突出了PCGD-TCL相关的诊断复杂性,强调了仔细的组织病理学检查和免疫表型特征分析的重要性。鉴于其侵袭性本质和快速播散倾向,早期识别PCGD-TCL对于启动适当的治疗干预至关重要。然而,PCGD-TCL的有效治疗选择仍然有限,该病通常预后不良。需要进一步研究以阐明驱动PCGD-TCL发病机制的潜在分子机制,识别新的治疗靶点,并改善患者预后。此外,提高临床医生和病理学家对PCGD-TCL临床表现和诊断标准的认识,对于促进这一具有挑战性的恶性肿瘤的及时诊断和管理至关重要。

展开英文摘要原文

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.

论文信息

作者
Shaker N、Blankenship H、Masatkar V、Niu S、Sangueza OP
第一作者单位
Department of Pathology, The Ohio State University Wexner Medical Center/James Cancer Hospital, Columbus, OH.United States
通讯作者单位
Department of Pathology and Dermatology, Wake Forest University, School of Medicine, Medical Center Boulevard, Winston-Salem, NC; and.United States
文献类型
病例报告
期刊
The American Journal of dermatopathology2024 Sep 1
原文标识
PubMed 39150181 · DOI 10.1097/DAD.0000000000002743