Clinico-pathologic conference: 18-month old boy with fever and severe respiratory infection.
An 18-month-old boy with severe combined immunodeficiency (SCID) due to an IL2-y-receptor defect had a successful engraftment following a related mismatched allogenic bone transplant. He subsequently developed post-transplantation lymphoproliferative disorder, with severe respiratory infection which resulted in death. The case presentation is followed by a discussion with differential diagnosis of the clinical findings, and then by a discussion of the pathology found and the implications of this diagnosis.
Pomputius
William F
WF
Department of Pediatrics, University of South Florida, College of Medicine, Tampa, Florida 33606, USA.
Lacson
Atilano
A
Pomerance
Herbert H
HH
D018951:Antigens, CD20 / Q000096:biosynthesis; D001402:B-Lymphocytes / Q000473:pathology; D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D003937:Diagnosis, Differential; D005260:Female; D005334:Fever / Q000150:complications; D006801:Humans; D017403:In Situ Hybridization; D007223:Infant; D008099:Liver / Q000473:pathology; D008232:Lymphoproliferative Disorders / Q000150:complications / Q000209:etiology; D008297:Male; D015375:Receptors, Interleukin-2 / Q000235:genetics; D012141:Respiratory Tract Infections / Q000401:mortality*; D016511:Severe Combined Immunodeficiency / Q000150:complications / Q000175:diagnosis / Q000628:therapy*
D018951:Antigens, CD20; D015375:Receptors, Interleukin-2