title: Outcome of hematopoietic stem cell transplantation in severe combined immune deficiency with central nervous system viral infection.
abstract: BACKGROUND
  Patients with severe combined immunodeficiency and preexisting viral pneumonitis formally had a poor outcome from hematopoietic stem cell transplantation. With inhaled steroid and antitumor necrosis factor alpha antibody treatment, results improved. The poor outcome of patients with viral central nervous system infection prompted this retrospective single center review.
  
  
  RESULTS
  Eight of 71 patients with severe combined immunodeficiency transplanted since 1987 were identified with viral central nervous system infection (adenovirus [1], cytomegalovirus [2], Epstein-Barr virus [2], parvovirus [1], varicella zoster virus [1], human herpesvirus 6 [1]). Nonspecific neurologic symptoms included drowsiness, irritability, head lag, fisting and floppiness. Later symptoms included unresponsiveness, apnea, posturing, hypotonia, hyperreflexia and seizures. All had neuroradiologic investigations. Only one had an initially normal computed tomography scan. Magnetic resonance image abnormalities included cerebral atrophy, basal ganglia changes, diffuse leukoencephalopathy, and multifocal mass lesions. Five patients had virus identified from cerebrospinal fluid by polymerase chain reaction and brain tissue examination from 3 patients identified human herpesvirus 6, adenovirus type 41 and varicella zoster virus. Three children remain alive, 2 received replete marrow and one remains untransplanted. Others who received T cell depleted marrow died of neurologic sequelae.
  
  
  CONCLUSIONS
  Outcome of viral central nervous system infection after hematopoietic stem cell transplantation for severe combined immunodeficiency is poor, particularly associated with T cell depleted marrow.
authors.affiliation: Paediatric Immunology Department, Newcastle General Hospital, Newcastle upon Tyne, UK.
authors.forename: Cathy
authors.initials: C
authors.lastname: Waruiru
authors.forename: Mary A
authors.initials: MA
authors.lastname: Slatter
authors.forename: Clive
authors.initials: C
authors.lastname: Taylor
authors.forename: Venkateswaran
authors.initials: V
authors.lastname: Ramesh
authors.forename: Terence J
authors.initials: TJ
authors.lastname: Flood
authors.forename: Mario
authors.initials: M
authors.lastname: Abinun
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Cant
authors.forename: Andrew R
authors.initials: AR
authors.lastname: Gennery
mesh_terms: D020805:Central Nervous System Viral Diseases / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D016511:Severe Combined Immunodeficiency / Q000150:complications* / Q000628:therapy*; D016896:Treatment Outcome