上呼吸道窦组织细胞增生伴巨大淋巴结病临床病理观察

目的 探讨上呼吸道窦组织细胞增生伴巨大淋巴结病(SHML)的临床病理学特征及诊断、鉴别诊断要点.方法 对1例上呼吸道SHML进行临床病理分析及免疫组化研究,并进行文献复习.结果 上呼吸道SHML的临床表现为鼻阻、鼻出血、声嘶、呼吸困难、喘鸣等.镜下病变组织中有淋巴细胞、浆细胞、中性粒细胞、嗜酸性粒细胞浸润,并可见特征性吞噬完整的淋巴细胞、红细胞的组织细胞.免疫组化显示组织细胞S-100、CD68( ),CD1a(-).结论 上呼吸道SHML是一种罕见的肿瘤,其诊断和鉴别诊断主要依靠病理组织学和免疫组化.     

皮肤伴巨大淋巴结病性窦组织细胞增生症临床病理观察

目的 探讨完全局限于皮肤的伴巨大淋巴结病性窦组织细胞增生症的临床病理特点及鉴别诊断.方法 对1例皮肤病变进行常规病理检查,伊红染色(HE)及免疫组化(S-100,CD68,Lys,Mac387,CD31,CD1a,ⅩⅢa因子和CD34)染色,光镜观察,分析其病理学特征、免疫表型及鉴别诊断.结果 组织学上病变主要在皮肤真皮层,低倍镜下见深蓝染区域与淡粉染区域相间(重要诊断提示性背景病变);在炎症背景中有纤维分割;细胞组成见大量的单核或多核的组织细胞及散在的淋巴细胞、浆细胞、中性粒细胞等浸润,同时巨噬性组织细胞内可见吞噬的小淋巴细胞.免疫组化显示巨噬性组织细胞S-100强(+),CD68和Lys(+),Mac387(+),CD31(+),不表达CD1a、ⅩⅢa因子和CD34.病理诊断:皮肤伴巨大淋巴结病性窦组织细胞增生症.术后随访5年,无局部复发或远处转移.结论 完全局限于皮肤的伴巨大淋巴结病性窦组织细胞增生症是很少见的非肿瘤性疾病,临床行为属于良性/潜在恶性,易被误诊为淋巴网织系统恶性肿瘤.明确诊断主要依靠其独特的组织病理学,并辅以免疫组化标记.     

窦组织细胞增生伴巨大淋巴结病误诊结核病1例分析

窦组织细胞增生伴巨大淋巴结病（SHML）是一种十分少见的淋巴结或结外的反应性病变。我院收治SHML患者曾误诊为结核病1例,最后经手术病理免疫组化确诊,现分析如下。     

窦组织增生症伴巨大淋巴结病1例报告并文献复习

目的 提高对窦组织增生症伴巨大淋巴结病(Rosai-Dorfman病)的临床表现和病理诊断的认识.方法 报道1例经淋巴结活检确诊Rosai-Dorfman病患者的临床资料并结合文献进行讨论.结果 患者肺CT平扫示双侧肺野内可见多发、弥漫性分布的大小不等的结节影,纵隔内可见多组肿大淋巴结影.淋巴结病理可见组织细胞、浆细胞和淋巴细胞并可见吞噬淋巴细胞现象,免疫组化S-100及CD68阳性.该病临床表现多样,易误诊.结论 Rosai-Dorfman病累及气道是罕见的淋巴结外病变,治疗以综合治疗为主,治疗后随访.     

Rosai-Dorfman病7例并文献复习

目的 探讨Rosai-Dorfman病的临床病理学特征、诊断及鉴别诊断,并复习相关文献。方法 回顾性研究攀枝花市中心医院2014—2023年诊断为Rosai-Dorfman病的病例,所有病例均由3位高年资病理审核医师复阅。结果 7例患者,年龄7～54岁,男性3例,女性4例,男女比例3∶4。发生部位：鼻腔、臀部、右上肢上臂外侧、左侧顶部颅内、颈6椎管内、右乳各1例,左大腿、下腹部均发生1例。临床表现因发生部位不同而表现不一。镜下形态大致相同,可见显著增生的组织细胞,周边见大量淋巴细胞、浆细胞浸润,成片的组织细胞与成片的淋巴浆细胞呈交替排列的暗区和亮区分布,高倍镜下可见“伸入现象”,即组织细胞内含有大量完整的淋巴细胞。免疫组化染色显示S-100、CD68、Cyclin-D1阳性,Langerin、AE1/AE3阴性。结论 Rosai-Dorfman病发病率低,发病年龄广,女性患者较多见,临床及影像学表现不典型,诊断主要依靠显微镜下特征及免疫组化染色结果。     

窦组织细胞增生症并巨大淋巴结病伴淋巴结梗死1例

<正>窦组织细胞增生症并巨大淋巴结病(sinus histiocytosis with massive lymphad-enopathy,SHML)是一种具有特殊临床和病理特点的组织细胞增生性疾病,临床罕见。2007年4月我科收治SHML伴淋巴结梗死1例,现报告如下     

组织细胞坏死性淋巴结炎53例临床病理特征分析

目的：探讨组织细胞坏死性淋巴结炎（HNL）的临床、病理特点。方法：回顾分析53例HNL的临床资料、病理特征。结果：临床以首发症状为颈部淋巴结肿大占86.79%、发热占13.21%。HNL病理组织学表现为多种形态的组织细胞、免疫母细胞及浆细胞样单核细胞增生,伴有程度不同的核碎片。病变区淋巴细胞以CD45RO阳性、组织细胞CD68阳性细胞为主。结论：HNL的临床病理特征复杂,病理组织学及免疫组化检查是确诊的依据,此病预后良好,但易误诊为恶性淋巴瘤、其他淋巴结非肿瘤性疾病,应引起注意。     

颈部淋巴结边缘区淋巴瘤累及脾脏并伴显著肉芽肿反应病理分析

目的探讨颈部淋巴结边缘区淋巴瘤(nodal marginal zone lymphoma,NMZL)累及脾脏并伴显著肉芽肿反应的临床病理学特征和鉴别诊断要点。方法回顾性分析1例颈部NMZL累及脾脏并伴显著肉芽肿反应临床资料,分析其病理组织学和免疫表型、基因检测特征。结果男,34岁,因低热、消瘦、盗汗16个月,全身淋巴结及脾脏增大14个月就诊,初步诊断为脾功能亢进行脾脏切除术。脾脏活检及免疫组织化学染色(免疫组化)示:脾脏组织内多个结节样结构,周边见较多小中淋巴样细胞与组织细胞混合存在,部分结节间见较多淋巴样细胞,小中淋巴样细胞浸润红髓。脾脏内结节区CD68和CD163阳性表达,结节周围有较多CD20阳性的小中淋巴样细胞,部分结节间区淋巴样细胞聚集融合,Ki-67阳性率30%。倾向低级别B细胞淋巴瘤。右颈部淋巴结活检示:淋巴结仅见少量残存淋巴滤泡,小中淋巴细胞弥散分布。免疫组化示:淋巴结内小中淋巴样细胞CD20、CD43阳性,Bcl-2(部分阳性),Ki-67阳性率约40%。淋巴结基因检测发现克隆性基因重排。最终诊断:右颈部NMZL累及脾脏并伴显著肉芽肿反应。结论肉芽肿病变可能掩盖淋巴瘤的形态学改变,临床病理诊断时除需注重特殊染色排除特殊感染或其他病因,还需仔细分析肉芽肿之间的淋巴细胞,综合临床、形态学、免疫组化及分子遗传学检查结果以排除淋巴瘤的可能。     

结外Rosai-dorfman病8例临床病理分析

目的探讨淋巴结外Rosai-dorfman病(RDD)的临床病理特征、诊断及鉴别诊断。方法对8例结外RDD患者进行HE染色和免疫组化检测,并复习相关文献。结果 8例患者均为结外病变,其中1例发生于胸腺。低倍镜显示病变主体由深染色区域及浅染色区域交错存在,呈"明暗"相间。高倍镜下可见组织细胞"伸入现象"。免疫表型:组织细胞S-100蛋白强(+),CD68(+),CD1α、CD21和CD56(-),淋巴细胞CD79a(+),浆细胞CD38和CD138(+)。结论结外RDD少见,尤其罕见发生于胸腺,组织形态学特征不明显,临床易误诊。结节状分布的结构特点、"明暗"相间的分布特征、组织细胞"伸入现象"及组织细胞CD68、S-100(+),而CD1a(-)有助于确诊。     

酷似淋巴瘤的淋巴结组织细胞增生性病变的诊断与鉴别诊断

目的分析淋巴结组织细胞增生症的临床病理特点及诊断与鉴别诊断.方法对1例以组织细胞增生为主要特点的淋巴结病变进行光镜观察、免疫组化染色、基因重排检测,并结合国内10余家医院的会诊结果和文献进行讨论.结果患者女性,43岁.左颈部淋巴结无痛性肿大1月余.光镜下窦组织细胞显著增生伴上皮样细胞团聚和散在少量霍奇金样细胞,该细胞CD68、Lys、α1AT（＋）,S-100、CD1α、CD3、CD30、CD15、ALK、MTB、EBV（-）,无Ig与TCR基因重排.结论由于淋巴结中增生的霍奇金样细胞有EBV感染,并呈CD68（＋）,S-100、CD1a和CD30（-）,无Ig和TCR基因重排,是否属感染相关的组织细胞增生值得考虑.     

颈部淋巴结窦组织细胞增生伴巨大淋巴结病的CT表现与鉴别诊断

目的分析颈部淋巴结窦组织细胞增生伴巨大淋巴结病(sinus histiocytosis with massive lymphadenopathy,SHML)的CT影像表现及相关临床表现,探讨该病的诊断和鉴别诊断。方法回顾性分析经临床与病理证实的7例颈部淋巴结SHML患者的临床和CT影像资料,男4例,女3例,年龄2~39岁,7例均行CT平扫及增强扫描。结果活检证实的13枚受累淋巴结的最长径范围在0.8~4.1 cm,平均2.1 cm。CT平扫密度均匀减低者10枚(76.9%),增强扫描11枚呈多环状或半环状强化(84.6%),9枚边缘清晰,4枚边界不清。结论当青少年男性颈部肿大淋巴结CT平扫密度均匀但增强呈多环或半环状强化时应想到SHML的可能,熟悉其CT表现有助于将其纳入鉴别诊断,结合临床、影像学及实验室检查有助于确诊。     

Intrathoracic Rosai-Dorfman disease with spontaneous remission: a clinical report and a review of the literature

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare non-neoplastic disease that is characterized by a proliferation of histiocytes mostly in lymph nodes. However, the etiological mechanism of RDD still remains unclear. Intrathoracic manifestations of RDD are only observed in 2% of patients with RDD. Spontaneous remission was reported in about 20% of patients with RDD; however, there are no reports of an intrathoracic manifestation of RDD that showed a spontaneous remission within a short period of time. A 64-year-old Japanese female with dry cough and left chest pain was introduced to our hospital, and computed tomography revealed a pulmonary nodular lesion and enlarged mediastinal lymph nodes. The bronchial specimen obtained from the abnormal mucosal lesion showed massive infiltration of histiocytes underneath the bronchial epithelium and emperipolesis, a typical pathological finding in RDD, which is characterized by the presence of histiocyte-like cells engulfing intact lymphocytes. These histiocytes were positive for S-100 (one of the known positive markers of RDD) and for CD68 (a marker for various cells of the macrophage lineage). All these findings are consistent with the diagnosis of RDD. These radiological and endoscopic findings spontaneously resolved within four months without any treatment. In conclusion, clinicians should be aware of this disease as one of differential diagnoses of pulmonary nodules in combination with mediastinal lymph node enlargements, especially in order to differentiate it from primary lung cancer.     

Atypical lymphadenopathies of the head and neck

Atypical lymphadenopathies fail to achieve the morphologic criteria of a malignant neoplasm, but exceed the usual concepts of follicular, lymphoid, or sinus histiocytic hyperplasias. Rich cellular proliferations usually composed of prominent histiocytes, or immunoblasts, or both with or without a vascular scaffolding obscure the nodal architecture. Toxoplasmosis, infectious mononucleosis, zoster, and vaccination-induced lymphadenopathies are caused by infectious agents, dermatopathic lymphadenitis is associated with cutaneous disease, anticonvulsant pseudolymphoma occurs in individuals hypersensitive to anticonvulsants (usually phenytoin), and Chediak-Higashi syndrome is an inherited abnormality of lysosomal microtubule function; the causes of sinus histiocytosis with massive lymphadenopathy, giant lymph node hyperplasia, angioimmunoblastic lymphadenopathy, mucocutaneous lymph node syndrome, and this histiocytoses remain unknown. The clinical course of these abnormalities varies from self-limited acute diseases (viral lymphadenopathies, toxoplasmosis, dermatopathic lymphadenitis, and usually anticonvulsive lymphadenopathy) to protracted, but benign abnormalities (sinus histiocytes with massive lymphadenopathy, giant lymph node hyperplasia, and multifocal eosinophilic granuloma). The diagnosis of angioimmunoblastic lymphadenopathy, Chediak-Higashi syndrome, and mucocutaneous lymph node syndrome necessitates a guarded prognosis, for death or the advent of a malignant lymphoma may interrupt their clinical course. Acute disseminated histiocytosis, even though the proliferated cell lacks the cytologic criteria of malignancy, should be regarded and treated as a malignant neoplasm.     

Sonographic features of Rosai-Dorfman disease in the breast: A case report

Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) is an uncommon histiocytic disease of the lymph nodes. Extranodal presentation, especially in breast parenchymal tissue, is rare. A 54-year-old woman presented with a painful and palpable lump in the right breast. Ultrasonography revealed an irregular, indistinct, hypoechoic mass with a hyperechoic halo. Pathological analysis revealed proliferation of large histiocytes and stromal fibrosis with emperipolesis and positive immunoreactivity for S-100 and CD68. The clinical and radiologic manifestations of Rosai-Dorfman disease may vary, and differentiation from other inflammatory diseases and malignancies is challenging; thus, accurate pathological diagnosis plays an important role in appropriate management.     

组织细胞性坏死性淋巴结炎的临床病理分析

目的 探讨组织细胞性坏死性淋巴结炎(HNL)的临床病理特征.方法 复习10例HNL淋巴结活检标本的HE切片,并用免疫组化SP法检测病灶内细胞的免疫表型.结果 HNL组织学上副皮质区,尤其是滤泡间区有散在大小不等或融合病灶,由多种形态的组织细胞、转化的淋巴细胞和凋亡碎屑构成,缺乏中性粒细胞、浆细胞和(或)嗜酸性粒细胞反应.部分淋巴细胞表达CD45RO,组织细胞表达CD68,残留的生发中心细胞表达CD20.结论 淋巴结活检显示有多种组织细胞、转化T细胞以及凋亡碎片组成的病灶,不合并有粒细胞浸润,有利于HNL的诊断.

Abstract：

Objective To describe the clinicopathological features of histiocytic necrotizing lymphadenitis ( HNL). Methods Routine lymph node biopsies of 10 HNL cases were reviewed and their immunophenotyping were performed using S-P immunohistochemical staining. Results Histologically, HNL had discrete or integrated nodules variable in size in the paracortex,expecially in the interfollicular area,which were full of proliferating pleomorphic histocytes,transformed lymphocytes,and karyorrhectic debris without infiltration of the neutrophils, plasma cells and/or eosinophils. Immunohistochemistry revealed CD45RO + for transformed lymphocytes,CD68 +for histiocytes.and CD20 +for lymphocytes in the residual germinal centers. Conclusion The presence of pleomorphic histiocytes, transformed T-cells, and karyor-rhectic debris in the biopsy of lymph nodes, together with the absence of neutrophils support the diagnosis of HNL.     

鼻背部Rosai-Dorfman病临床病理观察

目的探讨结外Rosai-Dorfman病的临床病理特点及诊断、鉴别诊断要点。方法对1例鼻背部Rosai-Dorfman病的肿瘤组织进行临床病理、免疫组化分析。结果该病镜下表现为多量泡沫样组织细胞及弥漫散在的淋巴细胞和浆细胞,并可见淋巴细胞吞噬现象。组织细胞S-100和CD68( )。结论发生于鼻部的结外Rosai-Dorfman病罕见,须与鼻硬结病及Langerhans细胞组织细胞增生症等鉴别。     

组织细胞性坏死性淋巴结炎病理组织学分析

目的探讨组织细胞性坏死性淋巴结炎(HNL)的临床病理特征。方法复习36例HNL淋巴结活检标本的HE切片,并用免疫组化SP法检测病灶内细胞的免疫表型。结果HNL组织学上副皮质区,尤其是滤泡间区有散在大小不等或融合病灶,由多种形态的组织细胞、转化的淋巴细胞和凋亡碎屑构成,缺乏中性粒细胞、浆细胞和/或嗜酸性粒细胞反应。部分淋巴细胞表达CD45RO,组织细胞表达CD68,残留的生发中心细胞表达CD20。结论HNL形态学上显示由多种组织细胞、转化T淋巴细胞及凋亡碎片组成的病灶,不见中性粒细胞浸润。应与恶性淋巴瘤、淋巴结的非肿瘤性病变鉴别。     

A case of rosai-dorfman disease with highly elevated serum ferritin

Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease is a rare disorder characterized by proliferation of distinctive histiocytes within lymph node sinuses and lymphatics, sometimes involving extranodal sites. However, clinical suspicion is difficult and there is also a lack of useful diagnostic markers for this disorder prior to histological confirmation. High elevation of serum ferritin is known to be a useful diagnostic marker for various hematologic diseases, including hemophagocytic lymphohistiocytosis and lymphoma. Here, we report a case of fever of unknown origin that presented along with highly elevated serum ferritin (5,780 ng/mL), and was finally diagnosed as Rosai-Dorfman disease by lymph node biopsy.     

Grey-scale and power Doppler sonography of unusual cervical lymphadenopathy

This study was undertaken to document the grey-scale and power Doppler sonographic features of cervical lymphadenopathy in Kikuchi's disease (histiocytic necrotising lymphadenitis), Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy), Sjogren's syndrome and systemic lupus erythematosus (SLE), which have not been reported in the literature. A retrospective review of the grey-scale and power Doppler sonograms of the cervical lymph nodes in nine patients was conducted (Kikuchi's disease, n = 3; Rosai-Dorfman disease, n = 1; Sjogren's syndrome, n = 1; SLE, n = 4). Lymph nodes were proven to be pathologic by fine-needle aspiration cytology (FNAC). On grey-scale ultrasound (US), lymph nodes were assessed by their distribution, size, shape, echogenicity and internal architecture. The vascular pattern of the lymph nodes was assessed with power Doppler sonography. US features of the lymph nodes were compared to those of metastatic and reactive nodes. In Kikuchi's disease, Rosai-Dorfman disease, Sjogren's syndrome and SLE, the distribution of lymph nodes is similar to that of reactive nodes. Most of the lymph nodes are enlarged with a maximum transverse diameter greater than or equal to 10 mm (83.3 to 100%). In Kikuchi's disease, lymph nodes have grey-scale and Doppler appearances similar to reactive nodes. However, lymph nodes in Rosai-Dorfman disease, Sjogren's syndrome and SLE show similar grey-scale and Doppler features to metastatic nodes. There is no specific US feature to characterise lymphadenopathy from these four miscellaneous causes. Definitive diagnosis should still be based on cytology and histology, and US can help in guiding FNAC for a more accurate cytologic examination.