Langerhans cell histiocytosis: Current concepts and treatments

"A Study of Adult Onset Generalized Eruptive Langerhans Cell Histiocytosis with Rare Association of Nail Pterygium"

“…It generally affects children with peak incidence at the age of 1-3 years and annual incidence of 4-5 cases per million. However, adult onset LCH is rare with annual incidence rate of 1-2 cases per million (1) .…”

“…Langerhans cell histiocytosis (LCH) is a rare disorder which is characterized by clonal proliferation of dendritic cells and mainly affects children under 10 years of age, although it can occur at any age, it is rarely reported in adults (1) . It comprises a wide range of clinical presentations depending upon the organs it affects.…”

Multisystem Langerhans Cell Histiocytosis: Case Report and Review of the Literature

“…The clinical course and biological behavior of LCH varies depending upon the sites and extent of involvement. [1] LCH is traditionally divided into two major categories; single system LCH and multisystem LCH defined as involvement of two or more organs at diagnosis with or without organ dysfunction. Bones are the most frequent site of this disorder, the flat bones are involved most frequently and among them the skull is commonest.…”

“…The clinical course of LCH is highly variable ranging from a self-healing solitary bone lesion to widely disseminated life-threatening disease. [1] In this paper a particular case of multisystem LCH presenting with bone and central nervous system (CNS) involvement is discussed with the existing literature.…”

Langerhans cell histiocytosis in acute leukemias of ambiguous or myeloid lineage in adult patients: support for a possible clonal relationship

“…It is a heterogeneous disease that may have a high mortality when multiple sites are involved or may resolve spontaneously as often occurs with single site disease. 1 Rarely, LC histiocytosis may be associated with various hematologic neoplasms including acute lymphoblastic and myeloid leukemias, other clonal myeloid neoplasms, and lymphomas. [2][3][4] Acute lymphoblastic leukemia is more often associated with LC histiocytosis in children, whereas lymphoma and acute myeloid leukemia are more common in adults, which may reflect the frequency of these diseases in the respective populations.…”