title: Treatment of refractory Langerhans cell histiocytosis (LCH) with a combination of 2-chlorodeoxyadenosine and cytosine arabinoside.
abstract: The combination of 2-chlorodeoxyadenosine (2-CDA) and cytosine arabinoside (Ara-C) has been shown to be effective in children with refractory Langerhans cell histiocytosis (LCH). We have treated 5 patients with recurrent LCH with 2-CDA/Ara-C chemotherapy and closely followed immune and hematopoietic function. These patients display a decline in the absolute CD4, CD8, and natural killer cell number, decrease in the CD4/CD8 ratio. Septic events, including pneumocystis infection were present after most of the treatment courses (15/21). These data suggest that 2-CDA /Ara-C, should be considered in resistant and relapsed pediatric patients with LCH with high-risk multiorgan involvement. Consequent profound prolonged combined immune deficiency and myelosupression should be anticipated.
authors.affiliation: The Valerie Fund Children's Center for Cancer and Blood Disorders, Monmouth Medical Center, Long Branch, NJ, USA. napollonsky@yahoo.com
authors.forename: Nataly
authors.initials: N
authors.lastname: Apollonsky
authors.forename: Jeffrey M
authors.initials: JM
authors.lastname: Lipton
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002675:Child, Preschool; D017338:Cladribine / Q000008:administration & dosage; D003561:Cytarabine / Q000008:administration & dosage; D006646:Histiocytosis, Langerhans-Cell / Q000000981:diagnostic imaging / Q000188:drug therapy*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007262:Infusions, Intravenous; D011859:Radiography; D012008:Recurrence; D015996:Survival Rate
chemical_list: D003561:Cytarabine; D017338:Cladribine