Osseous Involvement of Langerhans Cell Histiocytosis in Childhood : Case Reports

2018 ◽  
Vol 66 (3) ◽  
pp. 177-179
Author(s):  
Carla El Haber ◽  
Paul Henry Torbey
2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Nahid Reisi ◽  
Pouran Raeissi ◽  
Touraj Harati Khalilabad ◽  
Alireza Moafi

Abstract Background Langerhans cell histiocytosis (LCH) is a rare disease that originates from the uncontrolled proliferation and accumulation of bone marrow-derived immature myeloid dendritic cells. Dendritic cells are a type of histiocyte that play an important role in the human immune system and are found in the bone, skin, stomach, eyes, intestines, and lungs. Objective This systematic review aimed to collect and report published case reports of rare bone disease caused by LCH to avoid misdiagnoses or delays in diagnosis. Methods We systematically searched Scopus, PubMed, Embase, and Web of Sciences from August 1, 2000 to December 31, 2019. Studies reporting cases of LCH with rare bone involvement were included. Results We identified 60 articles including 64 cases. Of the identified cases, 31 (48.4%) involved children, and 33 (51.6%) involved adults. Additionally, 46.9% (30 individuals) were from Asian countries. The mean age of the children was 7.6 ± 4.3 years and that of the adults was 36 ± 12 years. The findings indicated that unifocal bone involvements were the most prevalent form of the disease (68.7%), and, overall, the skull and chest wall were the most commonly affected bones in both adults and children. The spine and long bones were the second most commonly affected bones in children, and the spine and jaw were the second most commonly affected bones in adults. Pain and swelling were the most frequent presenting signs among the investigated cases, and loss of consciousness, myelopathy, nerve palsy, visual loss, torticollis and clicking sounds were rare signs. Osteolytic lesions were the most frequent radiologic feature (62.5%), and intracranial hemorrhage, fluid–fluid level, dura and intracranial extension and pathologic fractures were rare radiological features. Total excision, curettage and observation in the unifocal group of patients and systemic chemotherapy in the other groups (i.e., multifocal and multisystem) were the most frequent management approaches. The recovery rates of the unifocal and multifocal groups were 77.3% and 81.8%, respectively, while that of the multisystem group was 55.5%. The rates of recurrence and mortality in the multisystem group were 11% and were higher than those in the other groups. Conclusions LCH is a rare disease that can affect any organ in the human body. However, bone is the most commonly involved organ, and rare bone involvements may be the first or only symptom of the disease due to the rarity of such lesions; a lack of familiarity with them may result in misdiagnosis or delayed diagnosis.


1996 ◽  
Vol 38 (2) ◽  
pp. 163-165 ◽  
Author(s):  
I. Mader ◽  
K. W. Stock ◽  
E. W. Radue ◽  
W. Steinbrich

2012 ◽  
Vol 02 (04) ◽  
pp. 77-79
Author(s):  
Jayaprakash Shetty K. ◽  
Ajith Kumar ◽  
Shantharam Shetty ◽  
Kishan Prasad H. L. ◽  
Chandrika Rao

AbstractLangerhans cell histiocytosis most commonly occurring in children, demonstrates a broad spectrum of clinical and radiologic features that may mimic those of infection as well as benign and malignant tumors. Osseous involvement is the most common manifestation. Recognition of the skeletal alterations is important so that the disease is considered as differential diagnosis. This disease is of unknown aetiology, non-specific clinical and imaging findings with diagnosis possible only on histopathological examination, will always pose a diagnostic challenge to the orthopaedic surgeon. Here by we are reporting two cases of eosinophilic granuloma that has presented with lytic lesion in the tibia and scapula. The clinico-radiological suspicion was either infection or tumour of the affected bones. Hence, core biopsy was done confirmed as eosinophilic granuloma on histopathology and immunohistochemistry.


Author(s):  
Dae-Seok Hwang ◽  
Jun Sang Lee ◽  
Uk-Kyu Kim ◽  
Hae Ryoun Park ◽  
Mi Heon Ryu ◽  
...  

F1000Research ◽  
2019 ◽  
Vol 8 ◽  
pp. 13
Author(s):  
Victoria Venning ◽  
Evelyn Yhao ◽  
Elizabeth Huynh ◽  
John W. Frew

Background: Cutaneous langerhans cell histiocytosis (LCH) is a rare disorder characterized by proliferation of cells with phenotypical characteristics of Langerhans cells. Although some cases spontaneously resolve, no consistent variables have been identified that predict which cases will manifest with systemic disease later in childhood. Methods: A systematic review (Pubmed, Embase, Cochrane database and all published abstracts from 1946-2018) was undertaken to collate all reported cases of cutaneous LCH in the international literature. This study was registered with PROSPERO (CRD42016051952). Descriptive statistics and correlation analyses were undertaken. Bias was analyzed according to GRADE criteria. Results: A total of 83 articles encompassing 128 cases of cutaneous LCH were identified. Multiple lesions were weakly associated with an increased length of survival (R=0.304 (p<0.05)), Worse prognosis was associated with internal organ involvement with a statistically significant chi squared statistic (χ2=14.96, 2DF p<0.001) and an elevated odds ratio ((OR)= 12.30 95% CI=2.67-56.74). Vesicular lesions (OR=10.8 95% CI=2.83-41.26), but not ulceration (OR=0.53 95% CI 0.12-2.05) were associated with greater risk of mortality. Conclusions: Congenital and neonatal LCH most commonly presents as multiple lesions in multiple anatomical sites at birth. Significant differences, including the associations of mortality with lesion morphology and number were seen in this neonatal cohort compared to overall pediatric LCH. These findings require validation in a large prospective cohort.


2018 ◽  
Vol 77 ◽  
pp. 147-151 ◽  
Author(s):  
Kazuhiko Kuwahara ◽  
Ko Kudo ◽  
Akiko Yashima-Abo ◽  
Kosuke Katayama ◽  
Keiko Kojima ◽  
...  

2010 ◽  
Vol 65 (5) ◽  
pp. 345-349 ◽  
Author(s):  
L. Decoster ◽  
K. De Braekeleer ◽  
C. Bourgain ◽  
D. Schallier

2019 ◽  
Vol 12 (3) ◽  
pp. 278-283
Author(s):  
Ilson Sepúlveda ◽  
Ignacio Mendoza ◽  
Romina Novoa ◽  
Geraldine Ayres ◽  
Nadia Inostroza ◽  
...  

2019 ◽  
Vol 12 (10) ◽  
pp. e231343 ◽  
Author(s):  
Abhishek Vaish ◽  
Raju Vaishya ◽  
Lokesh Kumar Singh ◽  
Vikas Kashyap

Langerhans cell histiocytosis (LCH) commonly occurs in children. It mimics infection and many benign and malignant tumours. This disease mainly involves the spine, skull and long bones, and its incidence is sporadic in the small bones of the foot and hand. We could not find any case reports with the involvement of a metatarsal bone, and hence, awareness about its possibility is essential to suspect it as a differential diagnosis of lytic lesions in the foot bones and therefore treat it judiciously. We have reported a case of a 35-year-old woman with spontaneous onset of pain over her right foot for the last year. An extensive curettage was performed, where the histology confirmed the features of LCH. Awareness about this entity and its differential diagnosis may help to clinch and early diagnosis and to treat effectively.


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