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Abstract：
UNASSIGNED: Cytophagic histiocytic panniculitis (CHP) is a rare panniculitis associated with systemic features characterized by the infiltration of subcutaneous adipose tissue by benign-appearing T lymphocytes and phagocytic histiocytes, mimicking hemophagocytic lymphohistiocytosis (HLH) and subcutaneous panniculitis-like T-cell lymphoma (SPTCL).
UNASSIGNED: To establish the clinicopathological features and response to treatment of CHP and evaluate the prognosis of patients and guide therapy based on the current state of knowledge.
UNASSIGNED: Clinical, laboratory, histopathological, and outcome data of 12 patients with CHP were retrospectively collected between 2009 and 2022.
UNASSIGNED: All the patients presented with plaques or nodules, mostly located in the lower extremities (11/12). Fewer cases involved systemic symptoms (9/12) and laboratory abnormalities (6/12), and none were positive for serum Epstein-Barr virus (EBV)-DNA. Histopathological examination revealed mixed septal and lobular inflammatory infiltration of histiocytes and lymphocytes. Large or atypical lymphocytes were rarely present (2/12). In some patients, varying proportions of plasma cells, neutrophils, and eosinophils were observed. The extent of histocytophagy was mild (9/12), moderate (2/12), and severe (1/12). HLH was not observed in any of our cases, none of which were fatal.
UNASSIGNED: The uniqueness of our study lies in the presence of neutrophil-rich dermal and subcutaneous infiltrates, associated with connective tissue disorders (CTD) and streptococcal infections. Our study reveals that EBV-negative CHP tends to a better prognosis than previously research, filling the gap in the much-needed details of CHP in the Chinese population. Moreover, CHP may present as a reactive process in combined primary diseases; further studies are required to validate these findings.
Cytophagic histiocytic panniculitis (CHP) is a rare panniculitis associated with systemic features characterized by the infiltration of subcutaneous adipose tissue by benign-appearing T lymphocytes and phagocytic histiocytes, also may be present in hemophagocytic lymphohistiocytosis and subcutaneous panniculitis-like T-cell lymphoma. The presence of neutrophil-rich dermal and subcutaneous infiltrates, associated with connective tissue disorders and streptococcal infections. In addition, EBV-negative CHP has a better prognosis than previously thought and provides knowledge of its prognosis in the Chinese population. With changes in the disease pedigree supported by the development of medical technology, CHP may present as a reactive process of a combined primary disease.
UNASSIGNED: To establish the clinicopathological features and response to treatment of CHP and evaluate the prognosis of patients and guide therapy based on the current state of knowledge.
UNASSIGNED: Clinical, laboratory, histopathological, and outcome data of 12 patients with CHP were retrospectively collected between 2009 and 2022.
UNASSIGNED: All the patients presented with plaques or nodules, mostly located in the lower extremities (11/12). Fewer cases involved systemic symptoms (9/12) and laboratory abnormalities (6/12), and none were positive for serum Epstein-Barr virus (EBV)-DNA. Histopathological examination revealed mixed septal and lobular inflammatory infiltration of histiocytes and lymphocytes. Large or atypical lymphocytes were rarely present (2/12). In some patients, varying proportions of plasma cells, neutrophils, and eosinophils were observed. The extent of histocytophagy was mild (9/12), moderate (2/12), and severe (1/12). HLH was not observed in any of our cases, none of which were fatal.
UNASSIGNED: The uniqueness of our study lies in the presence of neutrophil-rich dermal and subcutaneous infiltrates, associated with connective tissue disorders (CTD) and streptococcal infections. Our study reveals that EBV-negative CHP tends to a better prognosis than previously research, filling the gap in the much-needed details of CHP in the Chinese population. Moreover, CHP may present as a reactive process in combined primary diseases; further studies are required to validate these findings.
Cytophagic histiocytic panniculitis (CHP) is a rare panniculitis associated with systemic features characterized by the infiltration of subcutaneous adipose tissue by benign-appearing T lymphocytes and phagocytic histiocytes, also may be present in hemophagocytic lymphohistiocytosis and subcutaneous panniculitis-like T-cell lymphoma. The presence of neutrophil-rich dermal and subcutaneous infiltrates, associated with connective tissue disorders and streptococcal infections. In addition, EBV-negative CHP has a better prognosis than previously thought and provides knowledge of its prognosis in the Chinese population. With changes in the disease pedigree supported by the development of medical technology, CHP may present as a reactive process of a combined primary disease.
摘要：
■细胞吞噬性组织细胞性脂膜炎（CHP）是一种罕见的脂膜炎，与以良性T淋巴细胞和吞噬性组织细胞浸润皮下脂肪组织为特征的全身特征相关，模仿噬血细胞性淋巴组织细胞增多症（HLH）和皮下脂膜炎样T细胞淋巴瘤（SPTCL）。
■建立CHP的临床病理特征和对治疗的反应，并根据现有知识评估患者的预后并指导治疗。
■临床，实验室,组织病理学,回顾性收集了2009年至2022年期间12例CHP患者的结局数据.
■所有患者出现斑块或结节，大多位于下肢（11/12）。更少的病例涉及全身症状（9/12）和实验室异常（6/12），血清EB病毒(EBV)-DNA无阳性。组织病理学检查显示组织细胞和淋巴细胞的间隔和小叶混合炎症浸润。大的或非典型的淋巴细胞很少出现（2/12）。在一些患者中，不同比例的浆细胞，中性粒细胞，观察到嗜酸性粒细胞。组织细胞吞噬程度为轻度（9/12），中等(2/12),严重（1/12）在我们的任何病例中均未观察到HLH，没有一个是致命的。
■我们研究的独特性在于富含中性粒细胞的真皮和皮下浸润物的存在，与结缔组织疾病（CTD）和链球菌感染相关。我们的研究表明，EBV阴性CHP比以前的研究预后更好，填补了中国人口急需的卫生防护中心细节的空白。此外，CHP可能在合并的原发疾病中表现为反应过程；需要进一步的研究来验证这些发现。
细胞吞噬性组织细胞脂膜炎（CHP）是一种罕见的脂膜炎，与以良性T淋巴细胞和吞噬细胞浸润皮下脂肪组织为特征的全身特征相关，也可能存在于噬血细胞性淋巴组织细胞增生症和皮下脂膜炎样T细胞淋巴瘤中。富含中性粒细胞的真皮和皮下浸润物的存在，与结缔组织疾病和链球菌感染有关。此外,EBV阴性CHP的预后比以前认为的更好，并提供了中国人群的预后知识。随着医学技术的发展，疾病谱系的变化，CHP可表现为合并原发疾病的反应性过程。
■建立CHP的临床病理特征和对治疗的反应，并根据现有知识评估患者的预后并指导治疗。
■临床，实验室,组织病理学,回顾性收集了2009年至2022年期间12例CHP患者的结局数据.
■所有患者出现斑块或结节，大多位于下肢（11/12）。更少的病例涉及全身症状（9/12）和实验室异常（6/12），血清EB病毒(EBV)-DNA无阳性。组织病理学检查显示组织细胞和淋巴细胞的间隔和小叶混合炎症浸润。大的或非典型的淋巴细胞很少出现（2/12）。在一些患者中，不同比例的浆细胞，中性粒细胞，观察到嗜酸性粒细胞。组织细胞吞噬程度为轻度（9/12），中等(2/12),严重（1/12）在我们的任何病例中均未观察到HLH，没有一个是致命的。
■我们研究的独特性在于富含中性粒细胞的真皮和皮下浸润物的存在，与结缔组织疾病（CTD）和链球菌感染相关。我们的研究表明，EBV阴性CHP比以前的研究预后更好，填补了中国人口急需的卫生防护中心细节的空白。此外，CHP可能在合并的原发疾病中表现为反应过程；需要进一步的研究来验证这些发现。
细胞吞噬性组织细胞脂膜炎（CHP）是一种罕见的脂膜炎，与以良性T淋巴细胞和吞噬细胞浸润皮下脂肪组织为特征的全身特征相关，也可能存在于噬血细胞性淋巴组织细胞增生症和皮下脂膜炎样T细胞淋巴瘤中。富含中性粒细胞的真皮和皮下浸润物的存在，与结缔组织疾病和链球菌感染有关。此外,EBV阴性CHP的预后比以前认为的更好，并提供了中国人群的预后知识。随着医学技术的发展，疾病谱系的变化，CHP可表现为合并原发疾病的反应性过程。
