原发性皮肤γ/δT细胞淋巴瘤的诊断和治疗挑战:两个月内死亡的病例研究

Nada Shaker, H. Blankenship, Vaishali Masatkar, Shuo Niu, Omar P. Sangueza
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摘要

原发性皮肤γ/δT细胞淋巴瘤(PCGD-TCL)是原发性皮肤淋巴瘤中一种罕见但侵袭性极强的亚型。PCGD-TCL 具有诊断困难和预后不良的特点,其独特的临床和组织病理学特征使其有别于其他原发性皮肤淋巴瘤亚型。在此,我们报告了一名 75 岁男性的病例,他最初出现背部和双下肢多处红斑丘疹。最初的活组织检查提示为 CD30 阴性表型的原发性皮肤 T 细胞淋巴瘤(PCTCL)。然而,在两个月的间隔期内,病情迅速发展,表现为胸部和上肢皮肤广泛受累。再次进行皮肤活检时,发现真皮层有非典型淋巴细胞,但无表皮向性。免疫组化分析显示 CD3、CD5 和 CD4 阳性,T 细胞受体δ(TCR delta)表达,CD8 和 CD30 表达缺失。这些结果与 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措施,但患者的病情迅速恶化,最终导致他在确诊 PCGD-TCL 后一个月内死亡。该病例凸显了 PCGD-TCL 诊断的复杂性,强调了仔细进行组织病理学检查和免疫表型鉴定的重要性。鉴于 PCGD-TCL 的侵袭性和快速扩散倾向,早期识别 PCGD-TCL 对启动适当的治疗干预至关重要。然而,PCGD-TCL 的有效治疗方案仍然有限,而且该病通常预后不良。需要进一步开展研究,以阐明驱动 PCGD-TCL 发病机制的潜在分子机制,确定新的治疗靶点,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,对于及时诊断和治疗这种具有挑战性的恶性肿瘤至关重要。
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Navigating Diagnostic and Therapeutic Challenges in Primary Cutaneous Gamma/Delta T-Cell Lymphoma: A Case Study of Fatal Outcomes Within Two Months
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.
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