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患者男,41 岁。双侧腹股沟及左腰部肿块半年余,破溃2周。包块初起质软,逐渐增大变硬,疼痛。近2周包块表面出现破溃。血常规正常,血糖偏高。给予抗生素治疗,包块仍红肿,术中见包块位于皮下,与周围界限较清楚,但黏连严重,肿块内部分区域坏死。病理检查示真皮及皮下组织中肿瘤细胞弥漫成片排列,大部分细胞体积较大,细胞形态多样;胞浆较丰富、嗜酸性;胞核不规则形,较大,呈分叶状或有凹痕,有的可见一纵向核沟,核仁突出,可见大量多核巨细胞。免疫组化:瘤细胞S-100蛋白、CD1a和波形蛋白阳性,溶菌酶弱阳性,CD68部分细胞阳性;CD20、CD3、CD30、CKpan、Actin、EMA、AFP、LCA、CD21、HMB45均阴性。诊断Langerhans细胞肉瘤。患者经放疗联合化疗,疗效不明显。于诊断后一年死亡。 相似文献
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Sheila M. Valentín‐Nogueras Rachelle Seijo‐Montes Elena Montalván‐Miró Jorge L. Sánchez 《Journal of cutaneous pathology》2013,40(7):670-675
Primary cutaneous neoplasms of histiocytes and dendritic cells are rare. Langerhans cells are a subset of antigen‐presenting dendritic cells. Neoplasms of Langerhans cells are classified into cytologically benign Langerhans cell histiocytosis and cytologically malignant Langerhans cell sarcoma. Langerhans cell sarcoma is a rare entity characterized by multiorgan involvement and an aggressive clinical course. To date, only 30 cases of Langerhans cell sarcoma, including the present case, have been reported. We report a new case of Langerhans cell sarcoma that presented with multifocal cutaneous involvement. Diagnosis was done based on histopathological, immunohistochemical evaluation, as well as ultrastructural analysis identifying the presence of Birbeck granules. Our case represents a new case of this extremely rare, overtly aggressive neoplasm of Langerhans cells. Within 2 years of diagnosis, the patient developed metastatic disease and consequently died. Early recognition is important because of the tendency of Langerhans cell sarcoma to recur and metastasize. Therefore, ancillary techniques such as immunohistochemical and ultrastructural studies to confirm the diagnosis are very advantageous. 相似文献
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患者男,41岁.双侧腹股沟及左腰部肿块半年余,破溃2周.包块初起质软,逐渐增大变硬,疼痛.近2周包块表面出现破溃.血常规正常,血糖偏高.给予抗生素治疗,包块仍红肿,术中见包块位于皮下,与周围界限较清楚,但黏连严重,肿块内部分区域坏死.病理检查示真皮及皮下组织中肿瘤细胞弥漫成片排列,大部分细胞体积较大,细胞形态多样;胞浆较丰富、嗜酸性;胞核不规则形,较大,呈分叶状或有凹痕,有的可见一纵向核沟,核仁突出,可见大量多核巨细胞.免疫组化:瘤细胞S-100蛋白、CD1a和波形蛋白阳性,溶菌酶弱阳性,CD68部分细胞阳性;CD20、CD3、CD30、CKpan、Actin、EMA、AFP、LCA、CD21、HMB45均阴性.诊断Langerhans细胞肉瘤.患者经放疗联合化疗,疗效不明显.于诊断后一年死亡. 相似文献
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Alexandra Papoudou-Bai Amalia Vassou Leonidas Marinos Konstantina Papathanasiou Eleni Kapsali Panagiotis Kanavaros 《Journal of cutaneous pathology》2020,47(2):161-165
The phenomenon of histiocytic/dendritic cell sarcomas arising through transformation of a pre-existed lymphoproliferative disease is called transdifferentiation. Langerhans cell sarcoma transdifferentiating from chronic lymphocytic leukemia/small lymphocytic lymphoma is extremely rare and all the reported cases were localized in lymph nodes. We present a case of concurrent cutaneous localization of Langerhans cell sarcoma and chronic lymphocytic leukemia/small lymphocytic lymphoma, in which the chronic lymphocytic leukemia/small lymphocytic lymphoma preceded the development of the Langerhans cell sarcoma. A cutaneous lesion from a 63-year-old patient with a history of chronic lymphocytic leukemia/small lymphocytic lymphoma was biopsied. The histologic examination revealed a mixture of two cell populations infiltrating diffusely the dermis. The first was composed of small lymphoid cells with somewhat monotonous appearance and mild nuclear atypia positive for PAX5, CD79a, CD20, CD23, CD5, and LEF1. The second was composed of large cells with abundant cytoplasm and pleomorphic nuclei. These cells were positive for CD1a, CD207, and S100 protein and exhibited a high mitotic rate and a high MIB-1 immunostaining index. Therefore, two different entities, chronic lymphocytic leukemia/small lymphocytic lymphoma and Langerhans cell sarcoma, were detected in the same skin fragment. The patient died 3 years after initial diagnosis of chronic lymphocytic leukemia/small lymphocytic lymphoma. 相似文献
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朗格汉斯细胞组织细胞增生症1例 总被引:9,自引:7,他引:2
患儿6个月,生后2个月开始腹部,头皮,腹股沟部出现浅红色丘疹,头皮有脂溢性结痂。腹股沟、会阴区出现糜烂,局部发生溃疡。经病理及免疫组化检查证实为朗格汉斯细胞组织细胞增生症。 相似文献
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Furmanczyk PS Lisle AE Caldwell RB Kraemer KG Mercer SE George E Argenyi ZB 《Journal of cutaneous pathology》2012,39(6):644-650
Histiocytic/dendritic cell sarcomas are rare tumors, a few of which have been reported in association with B-cell lymphoma/leukemia. Isolated reports have documented identical immunoglobulin gene rearrangements suggesting a common clonal origin for both the sarcoma and the B-cell neoplasm from individual patients. We report a case of a 75-year-old male with hairy cell leukemia who subsequently developed Langerhans cell sarcoma 1 year after his primary diagnosis of leukemia. The bone marrow biopsy containing hairy cell leukemia and skin biopsies of Langerhans cell sarcoma were evaluated by routine histology, immunohistochemistry, flow cytometric immunophenotyping and PCR-based gene rearrangement studies of the immunoglobulin heavy chain and kappa genes. The hairy cell leukemia showed characteristic morphologic, immunohistochemical and flow cytometric features. The Langerhans cell sarcoma showed pleomorphic cytology, a high mitotic rate and characteristic immunohistochemical staining for Langerin, S100 and CD1a. There was no evidence of B-cell differentiation or a background B-cell infiltrate based on the absence of immunoreactivity with antibodies to multiple B-cell markers. Identical immunoglobulin gene rearrangements were identified in both the hairy cell leukemia and Langerhans cell sarcoma specimens. Despite the phenotypic dissimilarity of the two neoplasms, identical immunoglobulin gene rearrangements indicate a common origin. 相似文献
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Sanjiv Jain † Virendra N Sehgal ‡ Pramila Bajaj§ 《Journal of the European Academy of Dermatology and Venereology》2000,14(3):212-215
Nail changes in Langerhans cell histiocytosis are distinctly uncommon. Paronychial erythema, swelling and subungual pustules of the fingernails and toenails were cardinal, and were supported by diffuse as well as dense collections of mononuclear Langerhans cells evidenced by microscopic investigation. Oral administration of co-trimoxazole (800 mg sulphamethoxazole + 160 mg trimethoprim) every 12 h, 50 mg/d cyclophosphamide and 80 mg/d predinisolone were the mainstay of treatment, supported by scalp tar shampoo and local betamethasone lotion application. 相似文献
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Tzu-Chun Lin Po-Yuan Wu Tze-Yi Lin Su-Peng Yeh Shyh-chang Chen Tsong-Liang Lee 《Dermatologica Sinica》2011,29(3):101-105
Mycosis fungoides is a form of cutaneous T-cell lymphoma (CTCL). Malignant CD4+ T cells have been found to adopt the T-regulatory (Treg) cell phenotype and function. We present the case of a 66-year-old man diagnosed with mycosis fungoides that was progressing from the plaque to the tumor stage. The histopathological examinations showed that the Langerhans cell (LC) infiltration in the skin lesion of the tumor stage was greater than that in the patch/plaque stage; the tumor stage lesions resembled LC histiocytosis pathologically. The associations among LCs, apoptotic tumor cells, Treg CTCL cells, and relevant cytokines are complex. Treg CTCL cells produce the immunosuppressive cytokines interleukin-10 and transforming growth factor beta, which facilitate continuous recruitment of LCs and maintenance of long-term dendritic cell immaturity, thereby explaining the remarkable LC infiltration in the tumor stage samples from our patient. This phenomenon indicates that LCs accompanied by Treg CTCL cells may play an important synergistic role in the tumor progression. The development of immunotherapy directed against Treg CTCL cells and LCs overproduction and other immunosuppressive cytokines may be a potent useful adjuvant and worthy of further investigation. 相似文献
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患儿,女,1个月。头面部红色丘疹、结痂,逐渐增多1个月。皮肤科查体:头皮、额头、躯干、四肢散在黄豆至豌豆大红褐色至黄褐色丘疹、丘疱疹、结痂,部分皮疹少量鳞屑,不易刮除。皮损组织病理示:表皮细胞间水肿,单一核细胞移入,基底细胞液化变性,真皮浅中层较多单一核细胞浸润,细胞核大、胞浆丰富。DIF:表皮细胞间及基底膜带IgG、C3、IgM、IgA阴性。免疫组化:CD1a、S100、CD68弱阳性,Langerin+。未予治疗,随访1个月皮疹基本消退,无新发皮疹。 相似文献
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朗格汉斯细胞组织细胞增生症皮肤损害临床分析 总被引:1,自引:0,他引:1
目的:分析朗格汉斯细胞组织细胞增生症(LCH)的临床特点.方法:回顾性分析近10余年国内文献报道的918例诊断LCH的临床资料.结果:918例LCH中皮肤受累510例,占55.5%,以皮疹为首发症状的106例,占11.55%.临床上以湿疹样、脂溢性皮炎样或紫癜样皮疹多见.所有病例均合并其他系统损害,以发热(52.07%),肝肿大(52.29%)、脾肿大(48.26%)、骨损害(38.78%)、肺部损害(36.16%)最为常见.在918例LCH中,男593例,女317例,性别不详8例,最小出生4h,最大65岁,1岁以下占19.17%.皮肤活检151例.结论:皮肤是LCH常累及器官,对于1岁以下的小儿出现不典型湿疹样、脂溢性皮炎样或紫癜样皮疹应警惕LCH的可能,应进一步完善各项检查以明确诊断. 相似文献
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Rafiq Andani Ivan Robertson Kelli P. A. MacDonald Simon Durrant Geoffrey R. Hill Kiarash Khosrotehrani 《Experimental dermatology》2014,23(1):75-77
Chronic graft‐versus‐host disease (cGVHD) is a common complication following allogeneic stem‐cell transplantation (SCT). Past studies have implicated the persistence of host antigen‐presenting cells (APCs) in GVHD. Our objective was to determine the frequency of host Langerhans cells (LCs) in normal skin post‐SCT and ask if their persistence could predict cGVHD. Biopsies of normal skin from 124 sex‐mismatched T‐cell‐replete allogenic SCT recipients were taken 100 days post‐transplant. Patients with acute GVHD and those with <9 months of follow‐up were excluded and prospective follow‐up information was collected from remaining 22 patients. CD1a staining and X and Y chromosome in‐situ hybridization were performed to label LCs and to identify their host or donor origin. At 3 months, 59 ± 5% of LCs were host derived. The density of LCs and the proportion of host‐derived LCs were similar between patients that did or did not develop cGVHD. Most LCs in the skin remained of host origin 3 months after SCT regardless of cGVHD status. This finding is in line with the redundant role of LCs in acute GVHD initiation uncovered in recent experimental models. 相似文献
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Langerhans cell histiocytosis (LCH) can be a difficult therapeutic problem. We present a 40-year-old woman with a 4-year history of LCH who was successfully treated with low-dose methotrexate (20 mg weekly). 相似文献
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Margaret H. Dennin BS Carly J. Roman MD Sarah L. Stein MD 《Pediatric dermatology》2018,35(2):e140-e141
Langerhans cell histiocytosis is exceedingly rare in premature infants, and the few cases reported suggest a poor prognosis with systemic involvement. We present a case of Langerhans cell histiocytosis limited to a single cutaneous lesion, presenting in a 27‐week‐gestation infant, which is the youngest gestational age of reported Langerhans cell histiocytosis cases. The lesion showed spontaneous resolution by 41 weeks corrected gestational age, and systemic involvement was absent, demonstrating a mild course of skin‐only Langerhans cell histiocytosis in a premature infant. 相似文献