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
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.