Langerhans cell histiocytosis (LCH): Guidelines for diagnosis, clinical work‐up, and treatment for patients till the age of 18 years

Abstract

These guidelines for the management of patients up to 18 years with Langerhans cell histiocytosis (LCH) have been set up by a group of experts involved in the Euro Histio Net project who participated in national or international studies and in peer reviewed publications. Existing guidelines were reviewed and changed where new evidence was available in the literature up to 2012. Data and publications have been ranked according to evidence based medicine and when there was a lack of published data, consensus between experts was sought. Guidelines for diagnosis, initial clinical work-up, and treatment and long-term follow-up of LCH patients are presented.

A Rare Presentation of Langerhans Cell Histiocytosis Tonsil Infiltration: Review of the Literature: Atypical Presentation of Langerhans Cell Histiocytosis

“…The distribution of affected organs shapes the prognosis of the disease [5]. The frequency of involvement of the bone is 80 %; skin, 25 %; the pituitary gland, 25 %; spleen, 15 %; liver, 15 %; the hematopoietic system, 15 %; lymph nodes, 5-10 %; and cranial involvement excluding the pituitary gland, 2-4 % [1]. The most commonly infiltrated bones are the skull, femur, lower jaw, pelvis, and vertebrae [6].…”

“…It usually affects the bone, skin, and pituitary gland, and occasionally affects the hematopoietic system, lymph nodes, and lungs [1]. LCH is most often found in the pediatric population and less commonly in adults [1]. In this paper, we present a case report of an adult patient with tonsil infiltration with LCH.…”

“…LCH presents with solitary organ involvement or as a multi-system disease. It usually affects the bone, skin, and pituitary gland, and occasionally affects the hematopoietic system, lymph nodes, and lungs [1]. LCH is most often found in the pediatric population and less commonly in adults [1].…”

A Rare Case Report on Langerhans Cell Histiocytosis(lch) in a 4 Yr Old Child.

“…Hematopoietic disease may be associated with secondary hemophagocytosis -a common finding in young patients who died from disease in a French study (Donadieu, personal communication). [6] Any child presenting with prolonged fever and bony swelling particularly of skull bone, we have to suspect LCH. However, neuroblastoma can present with similar features due to metastasis to skull bones.…”

The Adverse Prognostic Hallmarks in Identical Twins with Langerhans Cell Histiocytosis: A Clinical Report and Literature Review

“…Langerhans cell histiocytosis (LCH) is characterized by uncontrolled proliferation of Langerhans cells accompanying eosinophils (Haupt et al 2013). Also known as Langerhans cell granulomatosis or histiocytosis X, it is an idiopathic, monoclonal disease that primarily attacks children under 10 years old.…”