Spongiform encephalopathy following allogeneic cord blood transplant.
A 6 year old boy developed a fatal, rapidly progressive encephalopathy 5 months after a matched unrelated cord blood transplant. Autopsy findings revealed spongiform changes in his brain. The clinical course of this child's illness had many findings consistent with that of a transmissible spongiform encephalopathy (TSE). Pre-mortem and post-mortem studies failed to definitively determine an etiology. Spongiform encephalopathies include the TSEs and mitochondrial encephalopathies. Both should be considered in a post-hematopoietic stem cell transplant patient who develops a progressive encephalopathy when more common etiologies are not found.
O'Brien
Dennis
D
Pediatric Hematology/Oncology, Lutheran Children's Hospital, Fort Wayne, IN, USA. dobrien@lutheran-hosp.com
Klopfenstein
Kathryn
K
Gross
Thomas G
TG
Baker
Peter
P
Termuhlen
Amanda
A
D001921:Brain / Q000473:pathology; D002051:Burkitt Lymphoma / Q000601:surgery; D002648:Child; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D007414:Intestinal Neoplasms / Q000601:surgery*; D008279:Magnetic Resonance Imaging; D008297:Male; D017096:Prion Diseases / Q000134:cerebrospinal fluid / Q000209:etiology* / Q000473:pathology; D014184:Transplantation, Homologous