Clinical characteristics and outcomes of Langerhans cell histiocytosis at a single institution in Thailand: a 20-year retrospective study

Abstract

Background Langerhans cell histiocytosis (LCH) is a rare disease characterized by the various systems involved and clinical manifestations with a wide range of symptoms. Objectives To describe clinical characteristics, imaging, treatment, and outcomes of pediatric LCH at Phramongkutklao Hospital, Bangkok, Thailand. Methods We conducted a 20-year retrospective review of the medical records of patients diagnosed with LCH from birth to 21 years old from January 1, 1997, to December 31, 2016. Results In all, 14 patients with median age of 2.5 years were studied. Six (43%) patients had single-system (SS) LCH. Five patients (63%) with multisystem (MS) LCH (n = 8. 57%) had risk-organ involvement (RO+). All patients had plain X-ray imaging of their skull with 11 (79%) showing abnormal findings. Tc-99m bone imaging and fluorodeoxyglucose F18 (FDG) positron emission tomography (PET)-computed tomography (CT) demonstrated abnormal findings in 8 (89%) and 4 (29%) patients, respectively. The 5-year event-free survival (EFS) for patients with RO+ MS-LCH was less than that for those without risk-organ involvement (RO−) MS-LCH and SS-LCH (20% vs. 100%, P = 0.005). Hematological dysfunction, hypoalbuminemia, and conjugated hyperbilirubinemia may be worse prognostic factors for RO+ MS-LCH. Conclusion FDG-PET-CT might have a greater accuracy to detect LCH disease than conventional plain X-ray and Tc-99m bone imaging. RO+ MS-LCH has been encountered with relapse and poor outcomes. Hematopoietic involvement, hypoalbuminemia, and conjugated hyperbilirubinemia may be worse prognostic factors for RO+ MS-LCH.

Organic acidurias in Egyptian children: The urge for high‐risk screening

“…13 Metabolic acidosis, increased anion gap, hyperammonemia, and ketosis are the characteristic findings of OAs. 27 High lactate is a common finding in OAs; a result of the coenzyme A metabolism secondary interference. 12 In concordance with our findings, metabolic acidosis followed by lactic acidosis were the most common laboratory findings in patients with OAs.…”

“…Metabolic acidosis, increased anion gap, hyperammonemia, and ketosis are the characteristic findings of OAs 27 . High lactate is a common finding in OAs; a result of the coenzyme A metabolism secondary interference 12 .…”

“…11 Making a diagnosis of classic organic aciduria requires a high level of clinical suspicion and well equipped laboratories. 27 The laboratory techniques essential for the diagnosis of metabolic diseases such as GC-MS are expensive and far beyond the financial ability of developing countries. 14 However, our study confirmed the importance of integration of such technologies in specialized centers to achieve this diagnosis and to start prompt therapy.…”

“…Making a diagnosis of classic organic aciduria requires a high level of clinical suspicion and well equipped laboratories 27 . The laboratory techniques essential for the diagnosis of metabolic diseases such as GC–MS are expensive and far beyond the financial ability of developing countries 14 .…”

The Impact of PET/CT on Paediatric Oncology

“…2-[ 18 F]FDG PET/CT is recommended in the current National Comprehensive Cancer Network (NCCN) guidelines for staging and response assessment [80]. Multiple studies have demonstrated the utility of 2-[ 18 F]FDG PET/CT to detect more lesions than conventional imaging in multiple sites [81][82][83][84]. Jessop et al reported a sensitivity and specificity of 100% and 83%, respectively, with low false positive rates [79].…”