FATAL FAT-EMBOLISM SYNDROME IN A CHILD WITH UNDIAGNOSED HEMOGLOBIN S BETA(+) THALASSEMIA - A COMPLICATION OF ACUTE PARVOVIRUS B19 INFECTION/

Citation
Ka. Kolquist et al., FATAL FAT-EMBOLISM SYNDROME IN A CHILD WITH UNDIAGNOSED HEMOGLOBIN S BETA(+) THALASSEMIA - A COMPLICATION OF ACUTE PARVOVIRUS B19 INFECTION/, PEDIATRIC PATHOLOGY & LABORATORY MEDICINE, 16(1), 1996, pp. 71-82
Citations number
19
Categorie Soggetti
Pathology,Pediatrics
ISSN journal
10771042
Volume
16
Issue
1
Year of publication
1996
Pages
71 - 82
Database
ISI
SICI code
1077-1042(1996)16:1<71:FFSIAC>2.0.ZU;2-3
Abstract
Anemia, mental status changes, and fatal respiratory failure complicat ed a febrile illness in a previously healthy 14-year-old black female. At autopsy, widespread fat emboli and bone marrow necrosis were found . Hemoglobin electrophoresis on an antemortem, pretransfusion specimen revealed hemoglobin S/beta(+) thalassemia. Acute parvovirus B19 (PVB1 9) infection was suspected. Postmortem serum and a variety of paraffin -embedded tissues were assayed for PVB19DNA using the polymerase chain reaction (PCR). The expected PCR product was identified in the serum specimen and in paraffin-embedded sections of bone marrow, kidney, spl een, parathyroid, thyroid, adrenal, and gastrointestinal tract; lung, liver, ovary, fallopian tube, uterus, brain, heart, and pancreas were negative. PVB19 infection is highly contagious and may be rapidly fata l in children with hemoglobinopathies by several mechanisms, including fat embolism. Therefore, there exists the risk of multiple deaths, wi thin a family. The acute infection may be easily and expeditiously dia gnosed using serum or a variety of parraffin-embedded tissues.