Final Diagnosis -- Disseminated Fungal Sepsis (Complete Heart Block)


History of Short Gut Syndrome
Severe Jejunoileal Atresia, Treated with Total Parenteral Nutrition Since Birth
Status Post Multiple Intestinal Resections
Status Post Colostomy and Closure
Status Post Gastrostomy Tube Placement
Status Post Cholecystectomy Secondary to Cholelithiasis

Cholestatic Liver Disease Consistent with Hyperalimentation Effect, Diagnosed by Liver Biopsy
Hepatomegaly with Bridging Fibrosis, Cholestasis, and Steatosis

Status Post Small Intestinal Transplantation
Multiple Episodes of Cellular Rejection
Epstein-Barr Virus Infected Cells in Allograft Biopsies
Extensive Fibrous Adhesions of Allograft Small Intestine
Marked Thinning of Allograft Intestinal Wall with Mucosal Attenuation
All Intestinal and Vascular Anastomoses Intact
Polymorphous Posttransplant Lymphoproliferative Disease Involving
Allograft Small Bowel Serosa and Mucosa

Status Post Cadaveric Renal Transplantation
History of Renal Insufficiency
Vascular and Ureteroneocystostomy Anastomoses Intact
Acute Tubular Necrosis, Allograft Kidney
Small Native Kidneys (combined weight 75 grams, 165 expected)
Glomerulosclerosis, Tubulointerstitial Inflammation, Fibrosis, and Calcification, Native Kidneys

Disseminated Fungal Sepsis (Aspergillus fumigatus Cultured in Life and at Autopsy) Involving:
Atrial and Ventricular Myocardium
History of Cardiopulmonary Arrest 48 Hours Prior to Death
Left Lower Lung with Focal Hemorrhagic Consolidation
Allograft Small Bowel
Native Kidneys
Allograft Kidney

Diffuse Hemorrhage of Urinary Bladder Mucosa
Focal Hemorrhage of Gastric Mucosa
Focal Hemorrhage of Diaphragm

Splenomegaly (160 grams, 73 grams expected) with Lymphoid Depletion

Serous Ascites, 300 ml

Serosanguineous Pericardial Effusion, 35 ml

Changes Consistent with Chronic Asthma, Lungs, Bilateral

Autopsy Limited to Chest and Abdomen


The findings of fungal sepsis and lymphoproliferative disease, and absence of rejection, strongly suggest that the patient was overimmunosuppressed at the time of death. It is significant that while lymphoproliferative disease was not diagnosed in life, multiple allograft biopsies contained Epstein-Barr virus infected lymphocytes, as demonstrated by EBER in situ hybridization. The PTLD found at autopsy was predominantly serosal, and would not have been detectable by mucosal biopsy; the involvement of the mucosa by PTLD was rather spotty and would easily have been missed. At the time of the patient's admission for heart disease, sepsis was suspected, and tracheal cultures taken at that time were subsequently positive for Aspergillus fumigatus, the organism grown from various organs at autopsy. However, the extent of the patient's fungal infection and the extensive lymphoproliferative disease were not suspected in life.

Contributed by Paul S. Dickman, M.D. and Charles A. Richert, M.D.

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