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Outcome of hematopoietic stem cell transplantation in severe combined immune deficiency with central nervous system viral infection

Lookup NU author(s): Professor Mary Slatter, Dr Vankateswara Ramesh, Dr Terence Flood, Dr Mario Abinun, Professor Andrew Cant, Professor Andrew GenneryORCiD


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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 α 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. CONCLUSION: 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. © 2007 Lippincott Williams & Wilkins, Inc.

Publication metadata

Author(s): Waruiru C, Slatter MA, Taylor C, Ramesh V, Flood TJ, Abinun M, Cant AJ, Gennery AR

Publication type: Article

Publication status: Published

Journal: Pediatric Infectious Disease Journal

Year: 2007

Volume: 26

Issue: 2

Pages: 129-133

ISSN (print): 0891-3668

ISSN (electronic): 1532-0987

Publisher: Lippincott Williams & Wilkins


DOI: 10.1097/01.inf.0000250621.46742.b5

PubMed id: 17259874


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