title: Successful Treatment of Refractory Langerhans Cell Histiocytosis of the Choroid Plexus in a Child With Pulse Dexamethasone and Lenalidomide.
abstract: Refractory/relapsed Langerhans cell histiocytosis (LCH) has a difficult course with a guarded prognosis. Salvage treatments available are both expensive and highly toxic. On the basis of the pathophysiology of LCH, we used a novel protocol including pulse dexamethasone and lenalidomide in a child with refractory LCH involving the choroid plexus, which resulted in durable remission with minimal toxicity. The protocol was extrapolated from the FIRST trial for patients with multiple myeloma. We present the clinical course, treatment protocol, and outcome in this child, who is at present disease free and in remission 18 months posttreatment.
authors.affiliation: Departments of *Pediatric Hematology, Oncology and BMT †Radiology, Apollo Specialty Hospital, Chennai, Tamil Nadu, India.
authors.forename: Ramya
authors.initials: R
authors.lastname: Uppuluri
authors.forename: Sreejith
authors.initials: S
authors.lastname: Ramachandrakurup
authors.forename: Ravikanth
authors.initials: R
authors.lastname: Balaji
authors.forename: Divya
authors.initials: D
authors.lastname: Subburaj
authors.forename: Atish
authors.initials: A
authors.lastname: Bakane
authors.forename: Revathi
authors.initials: R
authors.lastname: Raj
mesh_terms: D002831:Choroid Plexus / Q000473:pathology*; D017338:Cladribine / Q000008:administration & dosage / Q000627:therapeutic use; D003561:Cytarabine / Q000008:administration & dosage / Q000627:therapeutic use; D003907:Dexamethasone / Q000008:administration & dosage / Q000627:therapeutic use*; D003919:Diabetes Insipidus / Q000209:etiology; D004351:Drug Resistance; D006646:Histiocytosis, Langerhans-Cell / Q000188:drug therapy*; D006801:Humans; D007223:Infant; D000077269:Lenalidomide; D008297:Male; D010904:Pituitary Gland, Posterior / Q000473:pathology; D011239:Prednisolone / Q000008:administration & dosage / Q000627:therapeutic use; D020551:Pulse Therapy, Drug; D012074:Remission Induction; D016879:Salvage Therapy; D019291:Skull Base / Q000473:pathology; D013792:Thalidomide / Q000008:administration & dosage / Q000031:analogs & derivatives* / Q000627:therapeutic use; D013904:Thoracic Vertebrae / Q000473:pathology; D014747:Vinblastine / Q000008:administration & dosage / Q000627:therapeutic use
chemical_list: D003561:Cytarabine; D017338:Cladribine; D013792:Thalidomide; D014747:Vinblastine; D003907:Dexamethasone; D011239:Prednisolone; D000077269:Lenalidomide