scholarly journals Case Report: Two Infant Cases of Langerhans Cell Histiocytosis Involving the Digestive Tract

2021 ◽  
Vol 9 ◽  
Author(s):  
Huan Wang ◽  
Yuesheng Wang ◽  
Ruifeng Wang ◽  
Xiaoqin Li
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Pediatric Patients ◽  
Early Recognition ◽  
Systemic Disease ◽  
Endoscopic Examination ◽  
Langerhans Cell ◽  
Gastrointestinal Involvement ◽  
Colorectal Mucosa ◽  
Colonic Biopsy ◽  
Uncertain Etiology

Langerhans cell histiocytosis (LCH) is a rare disease with uncertain etiology. Langerhans cell histiocytosis with involvement of the gastrointestinal tract is rare and is typically identified in pediatric patients with systemic disease. The present study reports two infantile cases of LCH who initially presented with diarrhea, hematochezia, and rash and were histologically missed on the original examination of the colonic biopsy sections. The diagnosis of LCH was later verified through immunohistochemistry. By combining our experience and previous reports, the multiple hemorrhagic spots of the colorectal mucosa and narrowness and erosion of the distal duodenum might be suggestive manifestations of gastrointestinal involvement in LCH on endoscopic examination. This might be helpful for the early recognition of the disease.

Isolated Langerhans Cell Histiocytosis Bone Lesion in Pediatric Patients

2015 ◽  
Vol 153 (5) ◽  
pp. 751-757 ◽  
Author(s):  
Aren Bezdjian ◽  
Abdullah A. Alarfaj ◽  
Namrata Varma ◽  
Sam J. Daniel
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Pediatric Patients ◽  
Bone Lesion ◽  
Langerhans Cell

scholarly journals Cutaneous Langerhans cell histiocytosis with gastrointestinal involvement treated with dabrafenib

2018 ◽  
Vol 4 (1) ◽  
pp. 95-97 ◽  
Author(s):  
Jacob R. Stewart ◽  
Era C. Murzaku ◽  
Titilola T. Sode ◽  
Katherine A. Gordon
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Gastrointestinal Involvement

Follow-up of pediatric patients treated by IVIG for Langerhans cell histiocytosis (LCH)-related neurodegenerative CNS disease

2014 ◽  
Vol 101 (2) ◽  
pp. 191-197 ◽  
Author(s):  
Shinsaku Imashuku ◽  
◽  
Naoto Fujita ◽  
Yoko Shioda ◽  
Haruyoshi Noma ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Pediatric Patients ◽  
Langerhans Cell ◽  
Cns Disease

scholarly journals Unrecognized oral manifestations of Langerhans cell histiocytosis which progressed to systemic disease

2006 ◽  
Vol 42 (1) ◽  
pp. 10-13 ◽  
Author(s):  
I. Alajbeg ◽  
V. Vucicevic Boras ◽  
R. Femenic ◽  
A. Cekic-Arambasin ◽  
M. Anicic ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Systemic Disease ◽  
Langerhans Cell ◽  
Oral Manifestations

scholarly journals Bone Scintigraphy in the Examining of Treatment-Naive Pediatric Patients With Langerhans Cell Histiocytosis

2020 ◽  
Vol 3 (1) ◽  
pp. 21-37
Author(s):  
A. S. Krylov ◽  
A. D. Ryzhkov ◽  
S. M. Kaspshik ◽  
M. A. Krylova ◽  
E. E. Stanyakina ◽  
...  
Keyword(s):  
Bone Scintigraphy ◽  
Langerhans Cell Histiocytosis ◽  
Pediatric Patients ◽  
Bone Destruction ◽  
Langerhans Cell ◽  
Whole Body ◽  
Unknown Etiology ◽  
X Ray ◽  
Treatment Naïve ◽  
Proven Case

Introduction: Histiocytosis is a heterogeneous group of rare diseases of unknown etiology. LCH is characterized by an abnormal proliferation of histiocytes (activated dendric cells and macrophages). Langerhans cell histiocytosis (LCH) is the most common form of histiocytosis, it is a potentially fatal diseases. Early detection of LCH plays an important role in its prognosis and outcome. However, the role of advanced methods of nuclear medicine in diagnosis of LCH is still to be researched. We have long-term experience in observing pediatric patients with LCH. Taking into the account the difficulty of diagnostic task for bone scintigraphy in identifying lytic bone destruction, we stated the following objective of the study.Purpose: Retrospective and prospective analysis of bone scintigraphy examinations of treatment-naive pediatric patients with LCH and calculate the diagnostic efficacy of bone scintigraphy. Material and methods: We analyzed 60 examinations of treatment-naive pediatric patients with proven case of LCH (2014-2019). The scanning was performed using whole body mode, 3 hours after intravenous injection of bone-seeking radiopharmaceutical 99mTc-MDP on Symbia E, T2 (Siemens, Germany). The median age was 5.6 years.Results: During examination we visualized 88 lesions with pathological level of accumulation of radiopharmaceutical in 60 patients. 84 with high level of accumulation (>120 %); 3 with slightly increased level of accumulation (100–120 %) and 1 with lower than normal level of accumulation (<100 %). The median level of accumulation of radiopharmaceutical was 268 %. Max level of accumulation was 1422 % (patient with subtotal involvement of femoral bone). Min — 60 % (patient with lytic destruction in orbital bone). During X-Ray we found out 97 pathological focuses of bone lytic destruction. In 3 patients with polyostotic form we found extra focuses on scintigraphy, which were confirmed with following examination and X-Ray. 8 lesions were not found on scintigraphy in 8 patients.Сonclusions: We evaluated diagnostic accuracy of bone scintigraphy with 99mTc-MDP in treatment-naïve pediatric patients with proven case of LCH. Sensitivity, specificity, NPV and PPV — 91.6, 50.0, 11.1, 98.6 %, respectively.

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.

scholarly journals Pediatric Langerhans Cell Histiocytosis (LCH) Clinical Outcome in Hospital Civil De Guadalajara, México

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4951-4951 ◽  
Author(s):  
Katterine Rojas Rodríguez ◽  
Veronica Soto ◽  
Carlos Rodriguez-Galindo ◽  
Paola M. Friedrich ◽  
Edwin Guzmán ◽  
...  
Keyword(s):  
Clinical Outcome ◽  
Langerhans Cell Histiocytosis ◽  
Disease Risk ◽  
Conflicts Of Interest ◽  
Systemic Disease ◽  
Giant Cells ◽  
Langerhans Cell ◽  
Induction Treatment ◽  
Multisystem Disease ◽  
Clonal Proliferation

Abstract Pediatric Langerhans cell histiocytosis (LCH) clinical outcome in Hospital Civil de Guadalajara, México. Introduction : LCH results from clonal proliferation of functionally and immunophenotyped inmature round Langerhans cells along with eosinophiles, machrophages, lymphocytes and ocasionally multinucleated giant cells (1). Its incidence is 2-10 cases by million of children below 15 yr in US (2). Our objective was to describe the clinical characteristics and treatment outcome of patients with LCH at Departement of Hematology-Oncology of Hospital Civil de Guadalajara México. Methods: It was a retrospective design and 41 pediatric patients below 18 yr were included. The diagnosis was corroborated by pathology and immunohistochemistry. Variables as age, gender, localised vs systemic disease, risk organ commitment, global survival (GS) and event free survival (EFS) were analysed. We used descriptive and inferencial statistics with SPSS program. Results: There were included 41 patients from January 1st 2012 to December 31st 2017. Relation male:female was 1.1:1. Mean presentation was localised disease (58%). Bone was the principal affected structure (34%) and it was 71% to be combined with lung, lymph node and CNS compromise. Risk organ commitment was presented in 32%, being more frecquent bone marrow and liver in 22% each one. Time induction treatment was equal or below 12 weeks in 66% of patients. The 25% of patients had reactivation of LCH, with similar lesions to the beggining in 19.5%. We found statistically significant differences between dead patients (DP) (14.6%) and not dead patients (NDP) (85.4%) in clinical presentation: localised (0% in DP vs 69% in NDP) and systemic disease (100% vs 31%) (p=0,003) and risk organ commitment (100% in DP vs 20% in NDP) (p=0,000). Median age of 13 vs 24 months was for DP and NDP respectively. Conclusion: Dead patients were younger than 13 months old, with systemic disease, and risk organ commitment. We found a later asking of medical advice in DP (6 months) vs NDP (2 months). Keys words: langerhans cell histiocytosis, multisystem disease, risk organ Figure. Figure. Disclosures No relevant conflicts of interest to declare.

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