ABSTRACT
The hemophagocytic lymphohistiocytosis (HLH) syndrome is a hyperimmune disorder characterized by lymphohistiocytic infiltrations, elevated cytokine levels in the blood, macrophage activation, and hemophagocytosis, frequently presenting with a febrile septic picture. This unusual disease is more common in infancy and childhood than adulthood. It is classified as primary or familial when a genetic defect is identified and secondary or acquired when triggered by certain infections, autoimmune disorders, or malignancies. If or when such patients expire, they typically do so within a hospital or under a physician's care and so such cases rarely come to the attention of forensic pathologists. We report on the unexpected deaths of two hospitalized adult cases of HLH brought to autopsy without a premortem diagnosis. Postmortem examination demonstrated marked hepatosplenomegaly and lymphadenopathy in association with hemophagocytosis. Although very uncommon HLH must be considered in infants, children, or adults who die unexpectedly with an undiagnosed septic presentation.
Subject(s)
Death, Sudden/etiology , Lymphohistiocytosis, Hemophagocytic/diagnosis , Adult , DNA, Viral/genetics , Epstein-Barr Virus Infections/diagnosis , Female , Fever/etiology , Forensic Pathology , Hepatomegaly/pathology , Herpesvirus 4, Human/isolation & purification , Humans , Hypertriglyceridemia/etiology , Hypoalbuminemia/etiology , Hyponatremia/etiology , Lymphatic Diseases/pathology , Male , Middle Aged , Multiple Organ Failure/etiology , Pancytopenia/etiology , Splenomegaly/pathologyABSTRACT
As part of a review of substance abuse deaths in Dutchess County, New York, the Dutchess County Medical Examiner's Office identified 3 cases of angiocentric systemic foreign-body granulomatosis discovered at autopsy. Our investigation disclosed that in all 3 cases patients surreptitiously injected crushed oral prescription medication. Of the patients, 2 obtained intravenous access through central venous lines, the other patient was found to have injected herself intramuscularly. Autopsy demonstrated lung abnormalities due to diffuse deposits of foreign material within and around vessels, associated with foreign-body granulomatous reaction. We also identified a systemic distribution of this foreign material deposited beyond the lungs in the brain, heart, kidneys, and spleen. We present these cases along with a review of the literature of systemic embolization of foreign material in previously documented cases of deaths due to parenteral abuse of oral medications.
