title: Toxic Epidermal Necrolysis-like Reaction After Hematopoietic Stem Cell Transplantation in Children.
abstract: This study report clinical course, etiology, management, and long-term outcome of children who developed toxic epidermal necrolysis-like reaction (TEN-LR) after allogeneic hematopoietic stem cell transplantation (allo-HSCT). We retrospectively collected children with TEN-LR occurring after allo-HSCT performed in 2 pediatric bone marrow units between 2005 and 2014. We identified 6 cases of TEN-LR of 322 patients (1.8%). Possible triggers of TEN included antibiotics, antiepileptics, antimycotics, and Mycoplasma infection. In 3 patients TEN-LR occurred concurrently with severe multiorgan acute graft versus host disease. The management of TEN included administration of high doses of intravenous immunoglobulins and steroids (n=6), anti-tumor necrosis factor (n=3), and plasmapheresis (n=3) and whenever possible, discontinuation of the potentially causative drugs. Four patients (66%) reached a complete clinical response of TEN-LR after a median of 11.2 days. Two children (34%) are presently alive, 1 with long-term ocular sequelae. TEN-LR is a potentially lethal complication that may occur after HSCT also in pediatric patients. In our experience, TEN-LR and acute graft versus host disease probably coexisted and an overlap between the 2 forms is suggested. The multidisciplinary approaches involving specialized nurses, hematologists, dermatologists, burn surgeons, and infectious disease specialists is crucial to treat these patients.
authors.affiliation: *Paediatric Haematology-Oncology Department, Stem Cell Transplantation Unit †Immuno-Haematology and Transfusion Medicine Service ‡Infectious Diseases Unit ∥Intensive Care Unit, Istituto G. Gaslini ¶Dermatology Clinic, S Martino IST Hospital, Genova §SCT Unit Santobono-Pausilipon Hospital, Napoli, Italy.
authors.forename: Maura
authors.initials: M
authors.lastname: Faraci
authors.forename: Stefano
authors.initials: S
authors.lastname: Giardino
authors.forename: Edoardo
authors.initials: E
authors.lastname: Lanino
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Morreale
authors.forename: Emilia
authors.initials: E
authors.lastname: Ghibaudo
authors.forename: Monica
authors.initials: M
authors.lastname: Francesia Berta
authors.forename: Marco
authors.initials: M
authors.lastname: Risso
authors.forename: Elio
authors.initials: E
authors.lastname: Castagnola
authors.forename: Mimmo
authors.initials: M
authors.lastname: Ripaldi
authors.forename: Andrea
authors.initials: A
authors.lastname: Moscatelli
authors.forename: Giovanni
authors.initials: G
authors.lastname: Ghigliotti
mesh_terms: D000293:Adolescent; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage; D008297:Male; D010956:Plasmapheresis; D012189:Retrospective Studies; D013262:Stevens-Johnson Syndrome / Q000209:etiology* / Q000628:therapy*; D014409:Tumor Necrosis Factor-alpha / Q000276:immunology
chemical_list: D000911:Antibodies, Monoclonal; D016756:Immunoglobulins, Intravenous; D014409:Tumor Necrosis Factor-alpha