Sinus histiocytosis with massive lymphadenopathy, a report of 2 cases in Chinese

Two Chinese patients with sinus histiocytosis and massive lymphadenopathy are reported. The results of enzyme and immunohistochemical studies are presented.     

婴儿窦组织细胞增生伴巨大淋巴结病一例

患儿男,10个月.2个月前无明显诱因颈部出现许多包块.并进行性增大,不伴有症状于2008年10月入院.体检:B超显示颈部淋巴结肿大.CT检查:双侧颈部大块软组织影,考虑为颈部占位病变,淋巴瘤可能性大.实验室检查:三项异常:C-反应蛋白水平明显升高(27.9 mg/L);血清总蛋白(56 g/L)及白蛋白(32.3 g/L),略有下降;各种淋巴细胞数量异常(T淋巴细胞为0.20、CD3+/CD4+为0.14、CD3+/CD8+为0.06、CD16+/CD56+为0.58).临床先后二次切除左右颈部肿大包块送活检.     

婴儿窦组织细胞增生伴巨大淋巴结病一例

患儿男,10个月.2个月前无明显诱因颈部出现许多包块.并进行性增大,不伴有症状于2008年10月入院.体检:B超显示颈部淋巴结肿大.CT检查:双侧颈部大块软组织影,考虑为颈部占位病变,淋巴瘤可能性大.实验室检查:三项异常:C-反应蛋白水平明显升高(27.9 mg/L);血清总蛋白(56 g/L)及白蛋白(32.3 g/L),略有下降;各种淋巴细胞数量异常(T淋巴细胞为0.20、CD3+/CD4+为0.14、CD3+/CD8+为0.06、CD16+/CD56+为0.58).临床先后二次切除左右颈部肿大包块送活检.     

Sinus histiocytosis with massive lymphadenopathy in Rwanda: report of eight cases with immunohistochemical and ultrastructural studies

The clinico-pathological, immunohistochemical and ultrastructural features of eight cases of sinus histiocytosis with massive lymphadenopathy (SHML) recorded in Rwanda from 1975 to 1980 are reported.
The main histopathological features were a massive enlargement of the lymph node sinuses which were filled with large histiocytes, a great number of which phagocytosed blood cells, especially lymphocytes, and an important proliferation of plasma cells in the medullary cords. The pathogenesis of this syndrome is discussed, particularly the role of immunological disorders challenged by a chronic, but non-specific, infectious state. The ultrastructural study revealed no micro-organisms and no Langerhans' granules. Immunohistochemical staining revealed two interesting features: the presence of immunoglobulins in the cytoplasm of the histiocytes indicating the presence of an immune phagocytosis and the absence of lysozyme and α₁-antichymotrypsin (α₁-AC) from the histiocytes. The significance of the latter phenomenon is still unknown.     

Sinus histiocytosis with massive lymphadenopathy: the first three cases reported in Thailand

Three cases of sinus histiocytosis with massive lymphadenopathy are reported in Thai children and is probably a first report in Thailand. They were clinically characterized by bilateral massive cervical lymphadenopathy. Other lymph node groups were also involved but no extranodal manifestations could be detected. Leukocytosis with neutrophilia, hypergammaglobulinemia and polyclonal gammopathy were common features. The characteristic histopathologic findings in the involved lymph nodes included pericapsular fibrosis, markedly dilatation of subcapsular and medullary sinuses which were filled with numerous histiocytes showing active phagocytosis of lymphocytes and lymphoplasmacytoid proliferation in paracortical strands and medullary cord. The disease characteristically has a benign protracted clinical course, and does not respond to either irradiation or chemotherapy. The etiology and pathogenesis of this disorder are unknown. The possible pathologic mechanisms include an abnormal response to a specific infectious process and an immune deficiency status.     

Sinus histiocytosis with massive lymphadenopathy. Multiple skull involvements.

Sinus histiocytosis with massive lymphadenopathy is a non-neoplastic self-limiting disease of the bone marrow stem cell origin. It is characterized by painless, bilateral cervical lymphadenopathy accompanied by fever, leukocytosis, elevated erythrocyte sedimentation rate and hypergammaglobulinemia. Extranodal involvement including bone is rare. The patient is a 45-year -old female with multiple punch out lesions on her skull. MRI findings included iso-signal intensity mass at the diploid space on T1 weighted image and on T2 weighted image, mild high signal intensity was obtained. Histologically, the lesion showed proliferation of histiocytes in the fibroblastic background with formation of reactive germinal centers and many plasma cells. The histiocytes show round nuclei and occasional nucleoli and abundant cytoplasms. In area, there is lymphocytophagocytosis. Immunohistochemically, the histiocytes were positive for S-100 protein and lysozyme.     

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity

This report reviews the clinical and pathologic features of 423 examples of sinus histiocytosis with massive lymphadenopathy (SHML) entered in a case registry, with special emphasis on extranodal manifestations of the disease. The most common sites of extranodal involvement in this population are skin, upper respiratory tract, and bone. However, SHML also can occur in a variety of other sites, including the genitourinary system, lower respiratory tract, oral cavity, and soft tissues. Involvement of kidney, lower respiratory tract, or liver was found to be a poor prognostic sign, and patients with associated immunologic disease often fared poorly. In general, prognosis has been found to correlate both with the number of nodal groups and with the number of extranodal systems involved by SHML. A complete bibliography of publications describing patients with SHML is included, and illustrations of the clinical, histopathologic, and radiographic features of the disease are provided.     

Sinus histiocytosis with massive lymphadenopathy--a review of seven cases

Sinus histiocytosis with massive lymphadenopathy (SHML) also called as Rosai Dorfman disease is a rare histiocytic proliferative disorder of unknown etiology. Histological features currently define it. Persistent painless lymphadenopathy due to expansion of sinuses infiltrated with benign histiocytes and plasma cells and emperipolesis are the characteristic features of SHML. Our study includes seven cases (5 nodal and 2 extranodal) of SHML over a 5-year period whose slides and blocks were reviewed. IHC was performed on the main lesion, from a panel of S100, CD68, LCA, CD20, CD3, CD30, CD43, bcl2, cytokeratin and epithelial membrane antigen. In our series we have work up available in 7 cases out of which a detailed follow-up is available in 5 patients. Out of these 5 patients, 4 have a stable disease, while one developed histiocytic sarcoma after a gap of four years.     

Sinus histiocytosis with massive lymphadenopathy and unusual extranodal manifestations

We describe a predominant extranodal involvement of unusual sites by sinus histiocytosis with massive lymphadenopathy (SHML). The patient first presented at 13 years of age with bilateral parotid gland enlargement and was discovered to have a large asymptomatic left renal mass and adjacent hilar adenopathy, histologically diagnostic of SHML. Following a left nephrectomy, he remained well with persistent parotid and lacrimal gland disease and, 20 years later, plaquelike meningeal involvement mimicking meningioma en plaque developed that was associated with reactive meningeal nests within the body of the lesion. At no time during the last 20 years has there been a recurrence of significant external nodal enlargement, and biopsy specimens of neck and intrathoracic lymph nodes have been nondiagnostic of SHML.     

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of two cases with fine-needle aspiration cytology

Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease is a rare but well-defined histiocytic proliferative disorder. There are only few reports or small series of cases on the fine-needle aspiration (FNA) cytologic features of this entity. Our first case was a 14-yr-old girl who presented with low-grade fever and neck swellings of 2 mo duration. FNA smears from the submandibular and cervical lymph nodes showed numerous histiocytes with evidence of lymphophagocytosis (emperipolesis) against a background of reactive lymphoid cells. The FNA cytodiagnosis was Rosai-Dorfman disease. The patient was put on steroids, with improvement. The second patient was a 3(1/2)-yr-old girl who presented with fever and left cervical lymphadenopathy. The initial FNA smear from an outside laboratory was reported as reactive hyperplasia. A repeat FNA smear showed numerous histiocytes with evidence of emperipolesis, mild nuclear enlargement and multilobation/multinucleation in a few histiocytes, and reactive lymphoid cells. The cytodiagnosis was Rosai-Dorfman disease. Review of the initial smear showed the Rosai-Dorfman (RD) cells, but in smaller number. We conclude that FNA cytology is a useful tool in the diagnosis of SHML, but the diagnosis may be missed at initial stages when characteristic cytomorphologic features are not well-developed.     

Sinus histiocytosis with massive lymphadenopathy Rosai-Dorfman: three unusual manifestations

We report on three exceptional courses of sinus histiocytosis Rosai-Dorfman. Patient one developed regional lymph-node disease subsequent to two independent malignancies in the right head and neck region. Patient two suffered from extensive extranodal disease with more than 100 mucocutaneous lesions over 17 years, which spontaneously resolved. Patient three showed exclusively extranodal disease, including bilateral conjunctival/scleral lesions, before he developed lung cancer. Our cases are unique for three reasons: the association of the disease with solid malignancies in two cases, the extent and persistence of exclusively extranodal disease in one patient and the appearance of thus far undescribed conjunctival/scleral lesions.     

Sinus histiocytosis with massive lymphadenopathy. Report of a case associated with necrosis

We describe a case of a 21-year-old man who had a generalized lymphadenopathy and arthritis. Inguinal and cervical lymph nodes showed changes typical of sinus histiocytosis with massive lymphadenopathy. Extensive coagulative necrosis of the intrasinusal histiocytes is described, a feature which, to our knowledge, is unreported previously.     

淋巴结血管内T细胞淋巴瘤1例报道及文献复习

目的　探讨血管内淋巴瘤 (IVL)的临床病理特征。方法　对 1例腹股沟淋巴结IVL临床、病理组织学及免疫表型进行观察分析并复习文献。结果　男性 31岁 ,不明原因高热伴消瘦 5 0天 ,右腹股沟直径 1cm淋巴结 1枚 ,B超示肝脏轻度增大 ,血LDH明显升高伴ESR及转氨酶轻度升高 ,外周血WBC 3 3× 10 7/L ,骨髓像、多种病原及各肿瘤相关抗原检测均无异常。病理活检 :腹股沟淋巴结大部分破坏 ,代之以大量扩张的中小血管 ,腔内充满大量异型淋巴样细胞 ,局部伴管壁、管周浸润并累及结外脂肪组织。瘤细胞免疫表型CD4 5、CD4 5RO、CD3阳性 ,CK、CD6 8、CD79α、CD2 0均阴性 ,血管壁及内皮细胞CD31、CD34阳性。行CHOP化疗后症状缓解 ,现仍在随访中。结论　IVL是一罕见的非霍奇金淋巴瘤 ,好发于中枢神经系统及皮肤 ,其他部位少见 ,绝大数为B细胞型 ,T型罕见 ,以浅表淋巴结活检确诊者尚无报道。临床表现有一定提示性 ,确诊靠组织病理学检查 ,部分病例对化疗敏感 ,但多数病例预后差     

Sinus histiocytosis massive lymphadenopathy syndrome: histogenesis of the hepatic lesion

Sinus histiocytosis massive lymphadenopathy (SHML) syndrome with hepatic involvement, occurring in a seven-year-old black female, is reported. Morphologic characterization of the hepatic lesion is accomplished utilizing conventional light, fluorescent and electron microscopy, and histochemical techniques and by comparing and contrasting the findings with those in cases of familial erythrophagocytic lymphohistiocytosis (FEL) and virus-associated hemophagocytic syndrome (VAHS). The histiocytic proliferation in the liver in SHML differs by showing: (a) an intralobular distribution with portal sparing; (b) marked steatosis; and (c) lipofuscinosis. The aforesaid intralobular distribution and the accompanying hypertrophy and hyperplasia of Kupffer cells, as well as commonalities of steatosis and lipofuscinosis, and, to a lesser extent, erythrophagocytosis and siderosis, suggest a histogenesis from Kupffer cells. The histochemical finding of fatty acid peroxides and both fluorescent microscopic and histochemical evidence of lipofuscin inclusions, a by-product of lipid peroxidation, in Kupffer cells provide at least a theoretical basis for both erythrophagocytosis and proliferation eventuating in intralobular histiocytosis.     

Sinus histiocytosis with massive lymphadenopathy. Occurrence in identical twins with retroperitoneal disease

Sinus histiocytosis with massive lymphadenopathy (SHML) was originally defined as a relatively specific benign pseudolymphomatous disorder. Although the etiology remains unknown, the spectrum of SHML has been expanded to include predominance of extranodal disease in some patients, clinically significant immunologic abnormalities in 10% of patients, and fatal outcome in 7% of patients. We report the rare occurrence of SHML in identical twins; to our knowledge, SHML in identical twins has been reported only once previously. The two patients described are also unusual because of the predominance of retroperitoneal disease with minimal peripheral adenopathy. After a seven-year clinical course, one twin died of extensive retroperitoneal disease, liver failure, bleeding diathesis, and seizure disorder. The other twin is alive after a six-year course of progressive retroperitoneal disease.     

T淋巴母细胞性淋巴瘤/髓系肉瘤合并朗格汉斯细胞组织细胞增生症的临床病理分析

目的探讨T淋巴母细胞性淋巴瘤(T-LBL)/髓系肉瘤(MS)合并朗格汉斯细胞组织细胞增生症(LCH)的临床病理学特征、免疫表型及预后。方法收集中山大学附属佛山医院和首都医科大学北京友谊医院2013年12月至2019年4月间6例T-LBL/MS合并LCH患者临床及病理资料,采用HE染色、免疫组织化学EnVision法、原位杂交法进行染色分析,检索文献并复习。结果6例患者中,男性2例,女性4例。4例为T-LBL合并LCH,1例为T-LBL/MS合并LCH,1例为MS合并LCH。患者年龄5~77岁,中位年龄59岁。3例为多发淋巴结内病变,另3例为多发淋巴结及皮肤/肝脾病变。5例显示淋巴结结构破坏,3例可见数个残留萎缩的滤泡。瘤细胞有2种形态,一种为体积中等大小的淋巴样细胞,呈片巢状分布。核圆形、卵圆形,这类细胞主要分布于残留的滤泡旁和副皮质区。另一种为组织细胞样细胞,体积大,胞质丰富淡染或嗜双色性。核卵圆形、不规则形,呈折叠状,主要分布于淋巴结边缘窦、髓窦和滤泡间区。所有病例的背景嗜酸性粒细胞浸润不明显。中等大小淋巴样细胞表现为末端脱氧核苷酸转移酶(TdT)、CD99、CD7阳性,不同程度表达CD34、髓过氧化物酶(MPO)、CD2、CD3,Ki-67阳性指数多在30%~50%之间。而组织细胞样细胞表现为CD1a阳性、S-100蛋白阳性、Langerin表达不一,不同程度表达CD163/CD68,不表达T和B细胞标志物,Ki-67阳性指数多在10%~20%之间。所有病例均无EB病毒感染。随访4例(随访时间6~63个月,中位时间18.5个月),其中1例死亡,3例带病生存。结论T-LBL/MS合并LCH是一种少见的混合型幼稚淋巴造血系统疾病,多发生于皮肤、淋巴结,临床进展凶猛,预后差,故充分认识同一组织内两种病变成分有助于精准诊断与治疗。     

Sinus histiocytosis with massive lymphadenopathy and Langerhans cell histiocytosis express the cellular adhesion molecule CD31

Context.-We investigated expression of the adhesion molecule CD31 in sinus histiocytosis with massive lymphadenopathy (SHML) and Langerhans cell histiocytosis (LCH) because (1) SHML and LCH cells express a variety of cellular adhesion molecules and (2) SHML has been characterized as a reactive histiocytic proliferation, and tissue macrophages (histiocytes) are known to express CD31. Objective.-The purpose of this study was to determine whether SHML and LCH cells express CD31 and whether dual staining with CD31 and S100 facilitates diagnosis of these disease states. Methods.-Formalin-fixed, paraffin-embedded archival tissues were immunohistochemically stained via the labeled streptavidin-biotin method using antibodies against CD31 and S100 protein after heat-induced epitope retrieval. Archival tissues included SHML (n = 2), LCH (n = 10), malignant melanoma (n = 5), sinus hyperplasia (n = 4), granulomas (n = 4), granular cell tumor (n = 6), and normal skin (n = 4). Results.-Normal Langerhans cells in the epidermis were CD31(-)/S100(+); neoplastic Langerhans cells in LCH were CD31(+)/S100(+). Histiocytes in granulomas and in sinus hyperplasia were CD31(+)/S100(-); abnormal histiocytes in SHML were CD31(+)/S100(+). S100(+) tumors (malignant melanoma and granular cell tumor) were CD31(-). Conclusions.-The spectrum of cell types that express CD31 is expanded to include SHML and LCH. We speculate that up-regulation of CD31 in neoplastic Langerhans cells contributes to the migratory capability of LCH cells. CD31 may be a useful nonlysosomal marker of macrophages and their neoplastic counterparts (true histiocytic sarcomas). An immunohistochemical staining panel that includes CD31 and S100 facilitates the diagnosis of SHML and LCH.