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.

악성 조직구증의 진단 및 치료 경험

이종태(J . T . Lee),이근석(K . S . Lee),김인호(I . H . Kim),방영주(Y . J . Bang),박선양(S . Y . Park),김병국(B . K . Kim),김노경(N . N . Kim),허대석(D . S . Heo) 대한내과학회 1996 대한내과학회지 Vol.51 No.4

N/A Objectives: Malignant histiocytosis is a rare, rapidly fatal disorder, characterized by systemic proliferation of abnormal histiocytes. Most patients present with fever, organomegaly, and pancytopenia, and die within a few months. Although malignant histiocytosis represents diagnostically an important histiocytic disorder, its pathogenesis, natural history, response to therapy are not well known. In Korea, there are few reports on malignant histiocytosis. The purpose of this investigation is to assess the clinical spectrum natural history, laboratory findings, histopathologic findings response to therapy of malignant histijiocytos is diagnosed at Seoul national university hospital. Methods: The clinical records and histologic materials from 29 cases of malignant histiocytosis diagnosed at Seoul national university hospital between 1985 and 1994 have been reviewed. The criteria for the histopathologic diagnosis of malignant histiocytosis were the presence of large, abnormal malignant histiocytes with frequent mistosis to be distinguished from reactive histiocytic proliferation. All histologic materials were fixed in formalin, embedded in paraffin, sectioned, and stained with hematoxylin and eosin. Bone marrow aspirates were stained with Jener-Giemsa. of 29 patients, 20 patients were treated with combination chemotherapy; CHOP in 8 patients, COPBLAM in 3 patients, BVP in 7 patients, C-MOPP in 1 patient, Pdplusvincristine in 1 patient. Results: The median age was 32 years, with a 2.2:1 male to female prepondance. Major physical findings included temperature elevation (93%), hepatomegaly (90%), splenomegaly (86%), jaundice (5%). Common laboratory findings were anemia (86%), thrombocytopenia (90%), splenomegaly (86%), jaundice (45%). Common laboratory findings were anemia (86%), thrombocytopenia (90%), leukopenia (72%), hypocalcemia (97%), abnormal liver function test (100%). Histologic features observed in bone marrow were increased histiocytes, atypical immature histiocytic infiltration with hemophagocytosis. Histologic features observed in lymph node were destruction of normal structure with infiltration and proliferation of immature histiocytes. Prominent features observed in liver were sinusoidal histiocytic infiltration. The response to treatment was encouraging. One patient achieved a completed remission (5%), 5 patients achieved a partial remission (25%), and overall response rates were 30%. Conclusion: The distinctive clinical and histologic findings warrant recognition and separation of malignant histiocytosis from other histiocytic and hematopoietic disorders and long-term survival is possible in some patients if treated early and aggressively with combination chemotherapy.

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.

A case of benign cephalic histiocytosis

( Hee Jae Park ),( Byeong Chang Ko ),( Jae Hong Oh ),( Seung Pil ),( Ham Sik Haw ),( Hai-jin Park ),( Mira Choi ) 대한피부과학회 2019 대한피부과학회 학술발표대회집 Vol.71 No.1

Benign cephalic histiocytosis (BCH) is a rare, benign, non-Langerhans cell histiocytosis seen in infants and young children. It is characterized by a self healing eruption of multiple yellow to red-brown papules on the head and neck, occasionally spreading to the trunk and extremities. Recent studies suggested that BCH showed overlapping clinical, histologic, and ultrastructural features with juvenile xanthogranuloma (JXG) and generalized eruptive histiocytoma, which indicated they may belong to a one wide spectrum of non-Langerhans histiocytosis. A 12-month-old boy presented with scattered erythematous-yellow papules on the face which had appeared 4 months ago. Mucosa, palms, and soles were spared. He was otherwise healthy and developmentally normal. BCH, JXG and verruca plana were considered as differential diagnoses. Histopathological examination showed the proliferation of epithelioid histiocytic cells with amphophilic cytoplasm and prominent nucleoli in the upper and mid-reticular dermis. Immunohistochemically, the histiocytes were positive for CD68 while lacking expression of S-100, which helps to confirm the diagnosis as benign cephalic histiocytosis. As a few BCH cases have shown transformation into JXG involving ocular sites, he was recommended the ophthalmologic examination which revealed no other abnormalities. As there have been only four cases published in Korean literature; herein we decide to report a rare case of BCH.

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.

A Recent Update on Histiocytic Disorder in Children: Focus on Diagnosis and Treatment

윤회수 대한소아혈액종양학회 2020 Clinical Pediatric Hematology-Oncology Vol.27 No.1

The histiocytosis is rare disorder characterized by the accumulation of macrophages, dendritic cells, or monocyte-derived cells in various tissues and organs of children and adults. Classifying histiocytic disorders is difficult and has changed over time as an understanding of the biology of these cells has evolved. The most recently revised 2016 WHO classification of histiocytosis and neoplasms of the macrophagedendritic cell lineages has proposed grouping this diverse group of over 100 clinical entities into five main groups based on clinical, histologic, and molecular relevance. Comprehensive genomic studies for histiocytosis have been described and our understanding of the pathogenesis and biology has increased over the past decade. These advances will be able to make precision medicine and targeted therapy possible in patients with histiocytosis. Among the histiocytosis, this review mainly focuses on the updated diagnosis and treatment of Langerhans cell histiocytosis (LCH) and hemophagocytic lymphohistiocytosis (HLH) in children.

양측성, 반복성 자연 기흉을 동반한 폐 조직구증 1예

송우건,양세훈 圓光大學校 醫科學硏究所 1999 圓光醫科學 Vol.15 No.2

Pulmonary histiocytosis X can be associated with disseminated histiocytosis X, but isolated case in the lung parenchyme is rare. Pulmonary histiocytosis X is a diffuse, smoking-related, chronic interstitial lung disease characterized by proliferation and infiltration by langerhans cell and eosinophils. The hallmark of this disorder is the electron microscopic finding of X-body or Birbeck granule that present in the lung of patient. Pulmonary histiocytosis X is common in young adult and shows variable clinical characteristics. It caused by unknown etiology. A 18 year-old man was reffered to our hospital because of pneumothorax. Plain chest X-ray and chest CT revealed thin-walled cysts of various sizes in the both lungs. Video-associated thoracoscopic lung biopsy was performed. Light microscopic examination showed proliferation and infiltration of langerhans cells. Immunohistochemically, Langerhans cells showed strong cytoplasmic staining with S-100 protein and with HLA-DR. He was treated with right pleurodesis, but pneumothorax recurred 14 days later. We reported a case of bilateral spontaneous pneumothoraces in 18 year old with pulmonary histiocytosis-X, which is confirmed by eosinophilic granuloma in Video-associated thoracoscopic lung biopsy.

종격동에서 발생한 Rosai-Dorfman 질병의 증례 -1례 보고-

최주원,오태윤,조은윤,장운하 대한흉부외과학회 2002 Journal of Chest Surgery (J Chest Surg) Vol.35 No.10

Extranodal Rosai-Dorfman Disease in Mediastinum - A Case Report - Sinus histiocytosis with massive lymphadenopathy는 Rosai-Dorfman 질환(이후 RDD)으로 불리는 조직구증 증후군의 드문 형태의 질환으로, 주로 소아나 청소년기에 발생하며 대부분 경부 림프절을 침범하여 무통성의 심한 림프절 종대를 특징적으로 보이는 질환이다. 림프절외 부위 침범은 약 25~43%의 환자에서 보고되며, 일부에서는 림프절의 병변없이 림프절외 부위 침범만을 보이기도 한다. 림프절외 질환에서 침범되는 기관은 안구와 부속기관 두경부, 상기도, 유방, 위장관, 중추신경계 등의 다양한 장기에서 보고되고 있다. 최근 저자들은 35세 남자환자에서 수술전 방사선학적 검사상 전종격동 종괴가 발견되어 임상적으로 침윤성 흉선종의 의진 하에 수술을 시행하였으며, 수술 후 조직검사에서 RDD 질환으로 확진된 1예를 경험하여 보고하고자 한다. 5.Eisen RN, Buckley PJ, Rosai J. Immunophenotypic characterization of sinus histiocytosis with massive lymphadenopathy(Rosai-Dorfman disease). Semin Diagn Pathol 1990;7: 74-82. 6.Andriko JA, Morrison A, Colegial CH, Davis BJ, Jones RV. Rosai-Dorfman disease isolated to the central nervous system: a report of 11 cases. Mod Pathol 2001;14:172-8.7.Govender D, Chetty R. Inflammatory pseudotumour and Rosai-Dorfman disease of soft tissue: a histological continuum? J Clin Pathol 1997;50:79-81.8.Runde J, Ebbecke H, Kurlemann G, Frosch M, Schuierer G. Sinus histiocytosis with massive lymphadenopathy with complete occlusion of the superior and inferior vena cava. Klin Padiatr 1997;209:39-42.9.Silvestre JF, Aliaga A. Cutaneous Sinus Histiocytosis and chronic uveitis. Pediatr Dermatol 2000;17:377-80.10.Lhr HF, Gdderz W, Wlfe T. Long-term survival in a patient with Rosai-Dorfman disease treated with Interferon-α. Eur J Cancer 1995;31A:2427-28.11.Colleoni M, Gaion F, Perasole A, Nelli P, Manente P. Evidence of responsiveness to chemotherapy in aggressive Rosai-Dorfman disease. Eur J Cancer 1995;31A:424.

성인에서 발견된 중추성 요붕증이 합병된 폐 랑게르한스 세포 조직구증-1예 보고-

유병수,이재웅,조태준,홍기우,김건일,이원용,김동규,전선영 대한흉부외과학회 2005 Journal of Chest Surgery (J Chest Surg) Vol.38 No.12

Langerhans Cell Histiocytosis (LCH) is a pathologic proliferation and infiltration of various organs by Langerhans’ cells of unknown cause. Incidence rate of one million parties 3~4 is seen in young child but the incidence is not sure in adult. Organ systems involved by LCH may include skin, ear, bone marrow, liver, spleen, lung, pituitary gland-hypothalamus and GI tracts. In case pituitary-hypothalamus axis are involved, diabetes insipidus happened. Primary Pulmonary Langerhans Cell Histiocytosis(PLCH) with uninvolvement of other organs is rare and accompanied diabetes insipidus is more rare. There are many cases of LCH with diabetes insipidus involve such as central nervous system except lung. PLCH accompany central diabetes insipidus is only 1 case. We report a case of PLCH that accompany central diabetes insipidus with literature investigation in Department of Thoracic and Cardiovascular Surgery, Hallym University. 랑게르한스 세포 조직구증(Langerhans Cell Histiocytosis)은 병적인 랑게르한스 세포의 과도한 증식과 침윤으로 생기는 병으로 병태 생리가 확실하게 밝혀져 있지 않다. 소아에서 백만명 당 3∼4명의 발생률을 보이나 성인의 경우 그 발생빈도가 확실치 않다. 피부, 귀, 골수, 간, 비장, 폐, 뇌하수체-시상하부, 위장관 등 거의 모든 장기가 침범되며 뇌하수체-시상하부 축이 침범되면 요붕증이 발생한다. 원발성 폐 랑게르한스 세포 조직구증이 타장기의 침범과 동반되는 경우는 드물며, 요붕증을 동반한 경우는 더욱 드물다. 국내에서 랑게르한스 세포 조직구증 중 요붕증을 동반한 예는 다수의 보고가 있었으나 중추신경계 등 폐 이외의 장기침범이었으며 원발성 폐 랑게르한스 세포 조직구증과 동반된 중추성 요붕증은 1예가 보고되었다. 저자는 중추성 요붕증을 동반한 폐 랑게르한스 세포 조직구증을 경험하였기에 문헌고찰과 함께 보고하는 바이다.

랑게르한스세포 조직구증의 치료성적

한지숙,이승태,김종윤,민유홍,고윤웅,유철주,김길영,김병수 大韓血液學會 2001 大韓血液學會誌 Vol.36 No.2