scholarly journals Adult Langerhans cell histiocytosis and immunomodulatory drugs: Review and analysis of thirty-four case reports

2019 ◽  
Vol 8 (1) ◽  
pp. 1-8
Author(s):  
Endri Mauro ◽  
Piero Maria Stefani ◽  
Filippo Gherlinzoni
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Case Reports ◽  
Langerhans Cell ◽  
Immunomodulatory Drugs

scholarly journals Unusual sites of bone involvement in Langerhans cell histiocytosis: a systematic review of the literature

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Nahid Reisi ◽  
Pouran Raeissi ◽  
Touraj Harati Khalilabad ◽  
Alireza Moafi
Keyword(s):  
Systematic Review ◽  
Dendritic Cells ◽  
Rare Disease ◽  
Langerhans Cell Histiocytosis ◽  
Case Reports ◽  
Langerhans Cell ◽  
The Other ◽  
Bone Involvement ◽  
Intracranial Extension ◽  
Pathologic Fractures

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.

Langerhans cell histiocytosis in monocygote twins: case reports

1996 ◽  
Vol 38 (2) ◽  
pp. 163-165 ◽  
Author(s):  
I. Mader ◽  
K. W. Stock ◽  
E. W. Radue ◽  
W. Steinbrich
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Case Reports ◽  
Langerhans Cell

scholarly journals Langerhans cell histiocytosis of the mandible: two case reports and literature review

2019 ◽  
Vol 45 (3) ◽  
pp. 167
Author(s):  
Dae-Seok Hwang ◽  
Jun Sang Lee ◽  
Uk-Kyu Kim ◽  
Hae Ryoun Park ◽  
Mi Heon Ryu ◽  
...  
Keyword(s):  
Literature Review ◽  
Langerhans Cell Histiocytosis ◽  
Case Reports ◽  
Langerhans Cell

scholarly journals Cutaneous neonatal Langerhans cell histiocytosis: a systematic review of case reports

F1000Research ◽  
2019 ◽  
Vol 8 ◽  
pp. 13
Author(s):  
Victoria Venning ◽  
Evelyn Yhao ◽  
Elizabeth Huynh ◽  
John W. Frew
Keyword(s):  
Systematic Review ◽  
Langerhans Cell Histiocytosis ◽  
Case Reports ◽  
Systemic Disease ◽  
Langerhans Cell ◽  
Multiple Lesions ◽  
Risk Of Mortality ◽  
Cochrane Database ◽  
Chi Squared ◽  
Worse Prognosis

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.

LANGERHANS CELL HISTIOCYTOSIS: TWO CASE REPORTS IN ADULTS AND REVIEW OF THE LITERATURE

2010 ◽  
Vol 65 (5) ◽  
pp. 345-349 ◽  
Author(s):  
L. Decoster ◽  
K. De Braekeleer ◽  
C. Bourgain ◽  
D. Schallier
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Case Reports ◽  
Langerhans Cell ◽  
Review Of The Literature

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
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Case Reports ◽  
Langerhans Cell ◽  
Osseous Involvement

Head and neck Langerhans cell histiocytosis: two case reports and review of the literature

2019 ◽  
Vol 12 (3) ◽  
pp. 278-283
Author(s):  
Ilson Sepúlveda ◽  
Ignacio Mendoza ◽  
Romina Novoa ◽  
Geraldine Ayres ◽  
Nadia Inostroza ◽  
...  
Keyword(s):  
Head And Neck ◽  
Langerhans Cell Histiocytosis ◽  
Case Reports ◽  
Langerhans Cell ◽  
Review Of The Literature

scholarly journals Langerhans cell histiocytosis of a metatarsal bone in an adult female

2019 ◽  
Vol 12 (10) ◽  
pp. e231343 ◽  
Author(s):  
Abhishek Vaish ◽  
Raju Vaishya ◽  
Lokesh Kumar Singh ◽  
Vikas Kashyap
Keyword(s):  
Differential Diagnosis ◽  
Langerhans Cell Histiocytosis ◽  
Case Reports ◽  
Metatarsal Bone ◽  
Langerhans Cell ◽  
Malignant Tumours ◽  
Foot Bones ◽  
Lytic Lesions ◽  
Small Bones ◽  
Spontaneous Onset

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.

Langerhans cell histiocytosis (eosinophilic granuloma) of the skull mimicking nummular headache. Report of two cases

Cephalalgia ◽  
2017 ◽  
Vol 38 (4) ◽  
pp. 794-797 ◽  
Author(s):  
Carlos Silva Rosas ◽  
Heather Angus-Leppan ◽  
Melchor Bruno Lemp ◽  
Juan Pablo Rozas ◽  
Alonso Humberto Quijada
Keyword(s):  
Surgical Resection ◽  
Clinical Features ◽  
Langerhans Cell Histiocytosis ◽  
Eosinophilic Granuloma ◽  
Case Reports ◽  
Primary Headache ◽  
Langerhans Cell ◽  
Nummular Headache

Background Nummular headache is a rare, recently described topographic headache defined by the circumscribed coin-shaped area of pain. It is classified as a primary headache. There is debate about whether it is due to a peripheral or central disturbance, and its relationship to migraine. Case reports We report two patients with presumed nummular headache secondary to Langerhans cell histiocytosis, both with resolution of their headaches after surgical resection. Conclusion Imaging in patients with clinical features of nummular headache is recommended, as this and other cases highlight that it may be symptomatic. There are no distinguishing clinical features to separate nummular headache from secondary mimics, and treatment of the underlying cause may be curative.

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