title: Successful treatment of refractory Langerhans cell histiocytosis with unrelated cord blood transplantation.
abstract: A 2-month-old girl presented for treatment with a diffuse rash, interstitial pneumonia, otorrhea, and lymphadenopathy. Skin biopsy confirmed Langerhans cell histocytosis by electron microscopy. After receiving multiple courses of chemotherapy, only marginal improvement was achieved, with progressive marrow and liver involvement. The decision was made to pursue a human leukocyte antigen-identical unrelated cord blood transplantation. Two years after transplant, the bone marrow was clear of Langerhans cell histocytosis and 100% donor engraftment. The poor prognosis of patients with an inadequate response to therapy and the presence of organ dysfunction (marrow and liver) substantiated the decision to pursue an unrelated cord blood transplantation.
authors.affiliation: Department of Pediatrics, Division of Hematology/Oncology/Blood and Marrow Transplant, University of Minnesota, Minneapolis, USA. nagar003@tc.umn.edu
authors.forename: R
authors.initials: R
authors.lastname: Nagarajan
authors.forename: J
authors.initials: J
authors.lastname: Neglia
authors.forename: N
authors.initials: N
authors.lastname: Ramsay
authors.forename: K S
authors.initials: KS
authors.lastname: Baker
mesh_terms: D001853:Bone Marrow / Q000473:pathology; D017338:Cladribine / Q000627:therapeutic use; D003131:Combined Modality Therapy; D016572:Cyclosporine / Q000627:therapeutic use; D003561:Cytarabine / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005047:Etoposide / Q000627:therapeutic use; D005260:Female; D005312:Fetal Blood / Q000166:cytology*; D006086:Graft vs Host Disease / Q000517:prevention & control; D018380:Hematopoietic Stem Cell Transplantation; D006646:Histiocytosis, Langerhans-Cell / Q000151:congenital / Q000188:drug therapy / Q000628:therapy*; D006801:Humans; D007231:Infant, Newborn; D015122:Mercaptopurine / Q000627:therapeutic use; D008727:Methotrexate / Q000627:therapeutic use; D008775:Methylprednisolone / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use; D012074:Remission Induction; D014184:Transplantation, Homologous; D014747:Vinblastine / Q000627:therapeutic use
chemical_list: D003561:Cytarabine; D017338:Cladribine; D014747:Vinblastine; D005047:Etoposide; D004317:Doxorubicin; D016572:Cyclosporine; D015122:Mercaptopurine; D011241:Prednisone; D008775:Methylprednisolone; D008727:Methotrexate