PDF(Pubmed)
Abstract：
UNASSIGNED: To examine the clinical and pathological features, laboratory markers, therapeutic options and risk factors indicating poor prognosis of hydroa vacciniforme-like lymphoproliferative disorder (HVLPD).
UNASSIGNED: Seven patients with HVLPD had their clinical and pathological data collected. Immunohistochemical staining, Epstein-Barr virus-encoded RNA (EBER) in situ hybridization experiments, T-cell receptor (TCR) gene rearrangement, RT-PCR tests and the Elisa assay were carried out.
UNASSIGNED: The main clinical manifestations were papulovesicular lesions and ulcers on the face, neck, or trunk. Five cases had systemic symptoms. Three of the deceased patients had significant facial edema, deep body necrosis, and ulceration. The pathological results demonstrated that lymphocytes infiltrated blood vessels and sweat glands in addition to the dermis and subcutaneous tissues. All patients tested positive for CD3 and EBER. Six cases tested positive for TCRβF1, but none tested positive for TCRδ. TCRγ monoclonal rearrangement, strongly positive expression of TIA-1 and a Ki67 proliferation index of 40% occurred in 3 fatal cases. When compared to the survival group, the plasma EBV DNA in the deceased group was considerably higher (P<0.05). IFN-γ and TNF-α cytokine levels in patients were higher than in the control group, particularly in the deceased group (P<0.05). The skin lesions on all patients recovered quickly underwent conservative care. Nonetheless, 3 patients passed away as the disease progressed in its latter stages.
UNASSIGNED: In our cases, the main infiltrating cells were T cells and the dominant lymphocyte subclass was αβT cells. A significant increase in lgE level, plasma EBV DNA, IFN-γ, and TNF-α cytokine levels, decreased hemoglobin level, strongly positive expression of TIA-1, high Ki67 proliferation index, and positive TCR gene rearrangement are all indicators of a poor prognosis.
UNASSIGNED: Seven patients with HVLPD had their clinical and pathological data collected. Immunohistochemical staining, Epstein-Barr virus-encoded RNA (EBER) in situ hybridization experiments, T-cell receptor (TCR) gene rearrangement, RT-PCR tests and the Elisa assay were carried out.
UNASSIGNED: The main clinical manifestations were papulovesicular lesions and ulcers on the face, neck, or trunk. Five cases had systemic symptoms. Three of the deceased patients had significant facial edema, deep body necrosis, and ulceration. The pathological results demonstrated that lymphocytes infiltrated blood vessels and sweat glands in addition to the dermis and subcutaneous tissues. All patients tested positive for CD3 and EBER. Six cases tested positive for TCRβF1, but none tested positive for TCRδ. TCRγ monoclonal rearrangement, strongly positive expression of TIA-1 and a Ki67 proliferation index of 40% occurred in 3 fatal cases. When compared to the survival group, the plasma EBV DNA in the deceased group was considerably higher (P<0.05). IFN-γ and TNF-α cytokine levels in patients were higher than in the control group, particularly in the deceased group (P<0.05). The skin lesions on all patients recovered quickly underwent conservative care. Nonetheless, 3 patients passed away as the disease progressed in its latter stages.
UNASSIGNED: In our cases, the main infiltrating cells were T cells and the dominant lymphocyte subclass was αβT cells. A significant increase in lgE level, plasma EBV DNA, IFN-γ, and TNF-α cytokine levels, decreased hemoglobin level, strongly positive expression of TIA-1, high Ki67 proliferation index, and positive TCR gene rearrangement are all indicators of a poor prognosis.
摘要：
■为了检查临床和病理特征，实验室标记,治疗选择和危险因素表明疫苗水瘤样淋巴增生性疾病（HVLPD）预后不良。
■收集了7例HVLPD患者的临床和病理资料。免疫组织化学染色,EB病毒编码RNA(EBER)原位杂交实验,T细胞受体(TCR)基因重排,进行RT-PCR测试和Elisa测定。
■主要临床表现为面部丘疹泡状病变和溃疡，脖子,或树干。5例有全身症状。其中三名死者有明显的面部水肿,身体深层坏死，和溃疡。病理结果显示，除真皮和皮下组织外，淋巴细胞还浸润血管和汗腺。所有患者的CD3和EBER检测呈阳性。6例患者的TCRβF1检测为阳性，但无1例的TCRδ检测为阳性。TCRγ单克隆重排,在3例死亡病例中，TIA-1强阳性表达和Ki67增殖指数为40％。与生存组相比，死亡组血浆EBVDNA明显增高(P<0.05)。患者IFN-γ和TNF-α细胞因子水平高于对照组,尤其是死亡组(P<0.05)。所有患者的皮肤病变恢复迅速，并接受保守护理。尽管如此，3名患者在疾病后期进展时去世。
■在我们的案例中，主要浸润细胞为T细胞，优势淋巴细胞亚类为αβT细胞。lgE水平显著提高，血浆EBVDNA，IFN-γ，和TNF-α细胞因子水平，血红蛋白水平降低，TIA-1强阳性表达，高Ki67增殖指数，和TCR基因重排阳性都是预后不良的指标。
■收集了7例HVLPD患者的临床和病理资料。免疫组织化学染色,EB病毒编码RNA(EBER)原位杂交实验,T细胞受体(TCR)基因重排,进行RT-PCR测试和Elisa测定。
■主要临床表现为面部丘疹泡状病变和溃疡，脖子,或树干。5例有全身症状。其中三名死者有明显的面部水肿,身体深层坏死，和溃疡。病理结果显示，除真皮和皮下组织外，淋巴细胞还浸润血管和汗腺。所有患者的CD3和EBER检测呈阳性。6例患者的TCRβF1检测为阳性，但无1例的TCRδ检测为阳性。TCRγ单克隆重排,在3例死亡病例中，TIA-1强阳性表达和Ki67增殖指数为40％。与生存组相比，死亡组血浆EBVDNA明显增高(P<0.05)。患者IFN-γ和TNF-α细胞因子水平高于对照组,尤其是死亡组(P<0.05)。所有患者的皮肤病变恢复迅速，并接受保守护理。尽管如此，3名患者在疾病后期进展时去世。
■在我们的案例中，主要浸润细胞为T细胞，优势淋巴细胞亚类为αβT细胞。lgE水平显著提高，血浆EBVDNA，IFN-γ，和TNF-α细胞因子水平，血红蛋白水平降低，TIA-1强阳性表达，高Ki67增殖指数，和TCR基因重排阳性都是预后不良的指标。
