랑게르한스세포 조직구증 20예의 병리학적 특징과 Langerin (CD207)의 면역화학염색의 발현 특이성

류영준,정회선,신봉경,김애리,김한겸,김인선,이현주,이상호 대한병리학회 2009 Journal of Pathology and Translational Medicine Vol.43 No.2

Background : Langerhans cell histiocytosis (LCH) is a well-known neoplastic disorder of Langerhans cells which has characteristic findings, however, LCH has not been adequately studied in Korea. Methods : We analyzed the clinicopathologic features of 20 patients with LCH who were diagnosed between 1997 and 2006 at the Korea University Guro and Anam Hospitals. Results : The M:F ratio was 3:1 and the age ranged from 2-60 years (mean, 23.8 years [4 in 1st decade, 6 in 2nd decade, 2 in 3rd decade, 5 in 4th decade and 3≥40 years of age). The cases were classified as unifocal unisystemic in 13 patients, multifocal unisystemic in 4 patients, and multifocal multisystemic in 3 patients. The bone was the most commonly involved organ (14), followed by lymph node (5), lung (2), skin (2) and ureter (1). The Langerhans cells were immunohistochemically stained with Langerin, CD1a, S-100 protein, and CD68. Langerin and CD1a were specific for Langerhans cells. Conclusions : The distribution of the involved organs in patients with LCH was similar to the distribution in Western countries,but lymph node involvement was more frequent, whereas lung involvement was less common. Langerin is considered to be a specific marker for Langerhans cells. Background : Langerhans cell histiocytosis (LCH) is a well-known neoplastic disorder of Langerhans cells which has characteristic findings, however, LCH has not been adequately studied in Korea. Methods : We analyzed the clinicopathologic features of 20 patients with LCH who were diagnosed between 1997 and 2006 at the Korea University Guro and Anam Hospitals. Results : The M:F ratio was 3:1 and the age ranged from 2-60 years (mean, 23.8 years [4 in 1st decade, 6 in 2nd decade, 2 in 3rd decade, 5 in 4th decade and 3≥40 years of age). The cases were classified as unifocal unisystemic in 13 patients, multifocal unisystemic in 4 patients, and multifocal multisystemic in 3 patients. The bone was the most commonly involved organ (14), followed by lymph node (5), lung (2), skin (2) and ureter (1). The Langerhans cells were immunohistochemically stained with Langerin, CD1a, S-100 protein, and CD68. Langerin and CD1a were specific for Langerhans cells. Conclusions : The distribution of the involved organs in patients with LCH was similar to the distribution in Western countries,but lymph node involvement was more frequent, whereas lung involvement was less common. Langerin is considered to be a specific marker for Langerhans cells.

하악골에 발생한 Langerhans Cell Histiocytosis 환아의 증례보고

윤현주,이제호,윤정훈,이종갑 대한소아치과학회 2001 大韓小兒齒科學會誌 Vol.28 No.1

Langerhans Cell Histiocytosis는 Langerhans Cell 의 비정상적인 증식을 특징으로 하는 질환이다. 임상적 양상은 단독 골병소에서부터 여러 장기를 침범하여 치명적인 경우까지 매우 다양하게 나타나며, 나이가 어릴수록, 침범된 장기의 수가 많을수록 예후는 좋지 않다. 악골의 경우는 전체 Langerhans Cell Histiocytosis 환자의 10~20%에서 나타나며, 주로 초기에 이환된다. 본 환아는 만 3세 때 하악골 전반에 걸친 동통으로 본 치과병원에 내원하여 조직학적 검사를 통해 Langerhans Cell Histiocytosis임을 확진 받은후 소아과에 의뢰되어 화학요법에 의해 치료되었다. 재발의 가증성이 있어 현재 주기적 검사중이며, 하악 좌측 제2소구치배의 상실로 인해 향후 교정치료 및 보철치료가 필요하리라 사료된다. 본 증례는 치과병원에서 Langerhans Cell Histiocytosis임을 확진한 후 조기치료를 시행해 주어 양호한 결과를 얻었기에 보고하는 바이다. Langerhans Cell Histiocytosis(LCH) is characterized by proliferation of Langerhans cells. The clinical manifestation varies from solitary bone lesion to multi-system, life threatening disorder. The younger the patient is and the more system is involved, the worse the prognosis is. The jaw is involved 10~20 percent of all LCH and it is involved usually in early stage of LCH. In this case the patient is three years old girl who suffered from pain of whole mandibular body and histological examination confirmed the dignosis LCH. She is referred to pediatrics and managed with combined chemotherapy. Due to the possibility of recurrence, we follow up the girl and she need orthodontic and prosthodontic treatment in the future because of the loss of lower left 2nd premolar. We report this case because early recognized LCH in dental hospital result in good prognosis.

CASE REPORT : Pulmonary Langerhans Cell Histiocytosis in an Adult Male Presenting with Central Diabetes Insipidus and Diabetes Mellitus: A Case Report

( Yeun Seoung Choi ),( Jung Soo Lim ),( Woocheol Kwon ),( Soon Hee Jung ),( Il Hwan Park ),( Myoung Kyu Lee ),( Won Yeon Lee ),( Suk Joong Yong ),( Seok Jeong Lee ),( Ye Ryung Jung ),( Jiwon Choi ),( 대한결핵 및 호흡기학회 2015 Tuberculosis and Respiratory Diseases Vol.78 No.4

Pulmonary Langerhans cell histiocytosis is an uncommon diffuse cystic lung disease in adults. In rare cases, it can involve extrapulmonary organs and lead to endocrine abnormalities such as central diabetes insipidus. A 42-year-old man presented with polyphagia and polydipsia, as well as a dry cough and dyspnea on exertion. Magnetic resonance imaging of the hypothalamic-pituitary system failed to show the posterior pituitary, which is a typical finding in patients with central diabetes insipidus. This condition was confirmed by a water deprivation test, and the patient was also found to have type 2 diabetes mellitus. Computed tomographic scanning of the lungs revealed multiple, irregularly shaped cystic lesions and small nodules bilaterally, with sparing of the costophrenic angles. Lung biopsy through video-assisted thoracoscopic surgery revealed pulmonary Langerhans cell histiocytosis. On a follow-up visit, only 1 year after the patient had quit smoking, clinical and radiological improvement was significant. Here, we report an uncommon case of pulmonary Langerhans cell histiocytosis that simultaneously presented with diabetes insipidus and diabetes mellitus.

Non-Langerhans cell histiocytosis: generalized eruptive histiocytosis and benign cephalic histiocytosis

( Seon Gu Lee ),( Seung Hui Seok ),( Ji Hae An ),( In Jae Jeong ),( Hee Jung Lee ),( Moon Soo Yoon ),( Dong Hyun Kim ) 대한피부과학회 2018 대한피부과학회 학술발표대회집 Vol.70 No.1

Non-langerhans cell histiocytosis (NLCH) represents a broad group of different disorders characterized by the proliferation of histiocytes other than Langerhans cell. Generalized eruptive histiocytosis (GEH) and benign cephalic histiocytosis (BCH) belongs to NLCH, BCH is considered a limited form of the GEH. GEH is characterized by an asymptomatic, symmetric eruption of pink or dark red papules on the face, trunk and proximal extremities. BCH manifests as similar pattern of skin rashes distributed mainly on the head, face, neck and shoulders of infants and children. A 3-year-old girl visited our clinic with 2-year history of multiple 2-5mm sized yellowish papules on the face. A punch biopsy specimen revealed diffuse histiocytic infiltration in upper dermis. Immunohistochemically, the histiocytes are positive for CD68, negative for CD1a and S-100. BCH was diagnosed with consideration of histopathologic, immunohistochemical features and rashes limited to the face. A 16-year-old woman presented with multiple symmetrically distributed 3-10mm sized red to brown papules on the face, trunk and both extremities which developed 1 year ago. Histopathology also showed dermal infiltration of monomorphic histiocytes which were positive for CD68, but negative for CD1a and S-100. The patient was diagnosed with GEH. Both patients are under observation without other treatments. Herein, we report two cases of NLCH with review of literature.

두개골을 침범한 Langerhans' Cell Histiocytosis - 증례보고 -

손찬영,박상근,김한성,신형식,황용순,김상진,Son, Chan Young,Park, Sang Keun,Kim, Han Sung,Shin, Hyung Shik,Hwang, Yong Soon,Kim, Sang Jin 대한신경외과학회 2001 Journal of Korean neurosurgical society Vol.30 No.1

Langerhans' cell histiocytosis is a rare disease showing proliferation of histiocytes in multiple organ system. Bone lesions are the most common radiolologic manifestation of Langerhans' cell hysticytosis. Extraskeletal sites of Langerhans' cell hystiocytosis involvement include the skin, lymph nodes, thymus, lungs, central nervous system, liver, pancrease, spleen, and bowel. The authors have experienced a case of Langerhans' cell histiocytosis which involved the skull. A 3-year-old female presented with soft tissue mass on right periorbital area. Plain skull X-ray showed punched out bone lesion. Computed tomography showed non-enhancing osteolytic lesion on right frontal skull. Histologic findings of an excised mass revealed pathologic features of Langerhans' cell histiocytosis.

2세 이하에서 발생한 Langerhans 세포 조직구증

정양국(Yang-Guk Chung),김양수(Yang-Soo Kim),이승구(Seung-Koo Rhee),강용구(Yong-Koo Kang),박원종(Won-Jong Bahk),이안희(An-Hi Lee),박정미(Jung-Mi Park),김주영(Joo-Young Kim) 대한정형외과학회 2006 대한정형외과학회지 Vol.41 No.1

목적: 2세 이하에서 발생한 Langerhans 세포 조직구증의 임상적 특정과 치료결과를 분석하고 2세 이상에서 발생한 군과의 차이를 알아보고자 하였다. 대상 및 방법: 조직학적으로 확인된 2세 이하에서 발생한 Langerhans 세포 조직구증 14예에 대하여 주소, 발병 연령, 골 침범 부위 및 병소 수, 전신적 장기 침범 유무, 시행한 치료 및 최종 치료결과 등을 후향적으로 분석하고 같은 기간동안 치료받은 2세 이후에 발생한 14예와 비교하였다. 통계학적 검증은 Fisher Exact test를 이용하였다. 결과: 2세 이하에서 발생한 Langerhans 세포 조직구증은 2세 이상 군에 비해 진행된 병기를 보였고(2.5병기/1.1병기) 침범된 골부위 수가 많았으며(2.7병소/1.7병소) 전신 장기나 조직의 침범률이 높고(29%/0%) 더 빈번하게 화학요법이 필요하였다(9예/2예). 또한 빈번한 재발(0.5/0.3)과 낮은 완치율(57%/79%)의 경향을 보였으나 통계학적으로 유의한 차이는 없었다. 전체 62 골 병소 중 47 골 병변은 생검술 외의 특별한 수술적 치료 없이 경과 관찰이나 전신적 화학요법만으로 치유되었다. 결론: 2세 이하에서 발생한 Langerhans 세포 조직구증은 골 침범 부위가 많고 전신 장기나 조직의 침범률이 높으며 높은 재발의 경향을 보여 화학요법 등 보다 적극적인 치료가 요망되었으나 대부분의 골 병변은 수술적 치료 없이도 치유되었다. Purpose: To analyze the clinical characteristics and outcome of Langerhans' cell histiocytosis that developed in patients younger than 2 years and to compare them with those of older patients. Materials and Methods: The chief complaint, age of onset, site and number of bone involvements, systemic organ involvement, treatment and final outcome of fourteen cases of histologically proven Langerhans' cell histiocytosis that developed in patients younger than 2 years were analyzed retrospectively and compared with the results from those of fourteen older patients. The Fisher Exact test was used for statistical analysis. Results: Langerhans' cell histiocytosis that developed in patients younger than 2 years had a more advanced stage (2.5/1.1), multiple bone involvement (2.7/1.7 sites) and a higher rate of systemic organ involvement (29%/0%). It required chemotherapy more often (9/2 patients). There was also a trend of frequent relapse and a lower rate of complete healing, but this was not statistically significant. Among the 62 bone lesions, 47 were healed by simple observation or systemic chemotherapy without the need for surgical intervention. Conclusion: Langerhans' cell histiocytosis that developed in patients younger than 2 years showed multiple bone involvement, more systemic involvement and a high recurrence rate. It also required systemic chemotherapy more often. However most bone lesions were managed successfully without the need for surgical intervention.

림프절의 Langerhans세포 조직구증의 세침흡인 세포 소견 - 1예 보고 -

김은경,주종은,Kim, Eun-Kyung,Joo, Jong-Eun 대한세포병리학회 1997 대한세포병리학회지 Vol.8 No.2

Langerhans' cell histiocytosis is a relatively rare disorder of children, characterized by abnormal proliferation of Langerhans' cells. It usually presents as multiple osteolytic lesion with lymphadenopathy or cutaneous manifestations. We experienced a case of Langerhans' cell histiocytosis involving bone and lymph node, diagnosed by fine needle aspiration cytology of the lymph node. The patient was a 10-year old girl with left inguinal lymphadenopathy. Fine needle aspiration cytology from the lymph node disclosed very cellular smear consisted of Langerhans' cells, eosinophils and lymphocytes. The Langerhans' cells had eccentric oval to reniform shape nuclei with grooving and abundant pale cytoplasm. The diagnosis was confirmed later by histologic study of bone lesion.

A Case of Pulmonary Langerhans Cell Sarcoma Simultaneously Diagnosed with Cutaneous Langerhans Cell Histiocytosis Studied by Whole-Exome Sequencing

Kim, Si-Wook,Choi, Moon Ki,Han, Hye Sook,Song, Hyojin,Koh, Youngil,Son, Seung-Myoung,Lee, Ok-Jun,Lee, Ji Yeoun,Lee, Ki Man,Lee, Ki Hyeong,Kwon, Jihyun S.Karger 2017 Acta haematologica Vol.138 No.1

<P>Langerhans cell histiocytosis (LCH) and Langerhans cell sarcoma (LCS) are clonal proliferations of Langerhans-type cells. Unlike in LCH, the pathophysiology and clinical course of LCS are unclear due to its rarity. Here, we report the case of a 73-year-old male patient who was diagnosed with cutaneous LCH and pulmonary LCS at the same time. Pathological review of these 2 tumors revealed similar immunohistochemical findings. However, the tumor cells in LCS had more aggressive cytological features than those in LCH. Results of BRAF mutation analysis using real-time PCR were negative for both tumors. In whole-exome sequencing (WES), stop-gain mutations in TP53 gene were discovered only in LCS cells. The mechanism of development of LCS from various progenitor cells is currently unclear. According to the results of the WES study, changes in TP53 gene might have contributed to the malignant features of LCS.</P>

A case of solitary Langerhans cell histiocytosis

( Jee Yon Shin ),( Myeong Heon Chae ),( Ji Yeoun Lee ),( Tae Young Yoon ) 대한피부과학회 2019 대한피부과학회 학술발표대회집 Vol.71 No.1

Langerhans cell histiocytosis is caused by infiltration of the skin, and in some cases, other organs, by Langerhans cells. The spectrum of disease is broad. It can be presented with a solitary lesion and also presented with visceral and skin involvement. In childhood LCH, boys are slightly more often affected than girls. About 10% of children have single-organ disease involving only the skin, and 50% of children with multisystem LCH have skin involvement. A 17-year-old girl presented with solitary brownish nodule on the abdomen. It was occurred 3~4 months ago. Itching sense was mild and size change was not prominent. Histopathologic findings showed dermal infiltrate of Langerhans cells. On the immunohistochemistry, S-100 and CD1a was positive. There was no other skin lesion and no other organ involvement. Based on these findings, a diagnosis of solitary Langerhans cells histiocytosis was made. Herein, we report a rare case of solitary Langerhans cell histiocytosis in the Korean literatures.

Langerhans세포 조직구증의 세침흡인 세포학적 소견 - 1 예 보고 -

곽정자,진소영,이동화,Kwak, Jeong-Ja,Jin, So-Young,Lee, Dong-Wha 대한세포병리학회 1993 대한세포병리학회지 Vol.4 No.2

Langerhans cell histiocytosis or histiocytosis X is a disease of unknown etiology characterized by proliferation of mature histiocytes. While a few descriptions of the cytologic features of eosinophilic granuloma ocurring in the bone have been published, reports of cytologic findings of lymph node-based Langerhans cell histiocytosis are very rare. We report the cytologic findings of a case of Langerhans cell histiocytosis diagnosed by fine needle aspiration cytology from the left supraclavicular and right inguinal lymph nodes in a 65-year-old male. Cytologic smears showed characteristic reticuloendothelial cells which have elongated, folded, grooved nuclei and abundant pale cytoplasms. Particularly, nuclei were highly irregular and multilobated. A few mitotic figures were present. The cytologic diagnosis was confirmed by tissue biopsies from the left supraclavicular and right inguinal lymph nodes. Proliferation of histiocytes were also present in the skin. Immunohistochemistry for S-100 protein, vimentin, $\alpha1-antichymotrypsin$ and lysozyme showed positive staining. Electron microscopy disclosed Birbeck granules.