Contributed by Somak Roy, MD, Franklin A. Bontempo, MD, Larry Nichols, MD.
A 42-year-old white male presented with sudden onset of suprapubic and pelvic discomfort associated with gross hematuria. His vitals were stable on admission. A cystogram demonstrated a bladder of normal size and contour with no intravesical filling defect. Computed tomography (CT) revealed thickened bladder wall with possible infiltrating hematoma and obstruction of the right ureter with hydronephrosis.
Past medical history was significant for the following:
Table 1: Laboratory findings (coagulation profile) at initial diagnosis (8 years ago)
There was no significant family history of a coagulation disorder. He was an ex-smoker and denied alcohol use.
Table 2: Laboratory findings (coagulation profile) at time of admission
Table 3: Laboratory findings (other tests) at time of admission
HOSPITAL COURSE AND TREATMENT
He underwent cystoscopy with removal of intravesical clots and stenting of the right ureter. His renal function however, worsened. On day 3, he developed an occlusive thrombus in right cephalic vein, multiple ecchymotic patches on the anterior abdominal wall followed by extensive right femoral and popliteal vein clots with myonecrosis on day 6 of admission, requiring above knee amputation. This was followed by intramuscular hemorrhage in the gluteal muscles and facial skin gangrene on day 9. His coagulation profile, renal and pulmonary function worsened requiring mechanical ventilation. He ultimately died on day 11 of hospital admission.
GROSS AUTOPSY FINDINGS
The autopsy was performed on an already embalmed body. The pertinent gross findings included massive hemorrhagic necrosis of urinary bladder and prostate with large peri-vesical hematoma. Right coronary artery demonstrated in-stent thrombosis with 100% occlusion. There was moderate cardiomegaly with evidence of old myocardial infarction of the posterior left and right ventricular wall with severe fibrous endocardial thickening. Mucosal hemorrhage was noted in the hypopharynx, gastroesophageal junction and pylorus.
Figure 1. Gross image of facial skin demonstrating cutaneous gangrene (post embalming, with mortician's suturing).
Figure 2: Gross image of multiple cross sections of the right coronary artery demonstrating complete luminal occlusion. (post-electrochemical stent dissolution during autopsy)
Microscopic examination demonstrated thrombosis of small and medium sized arteries and veins in the bladder, prostate, lungs, renal glomeruli, peri-thyroidal vessels, hypopharyngeal and gastric mucosa and skin of the neck. The right coronary artery was occluded by intra-stent organized thrombosis. There was near total hemorrhagic infarction of the bladder and prostate, along with hemorrhagic infarction of right lower lobe lung, gastric and hypopharyngeal mucosal necrosis, severe ischemic cardiomyopathy and mild hepatic centrilobular ischemic changes.
Figure 3: Low power view of urinary bladder demonstrates transmural infraction of the bladder wall, complete denudation of the mucosa and thrombi in multiple vessels (Hematoxylin & eosin, 20x original magnification).
Figure 4: Hemorrhagic necrosis of prostate with thrombi in multiple vessels (Hematoxylin & eosin, 40x original magnification).
Figure 5: Section of the right kidney demonstrating multiple microthrombi (black arrows) in the glomerular capillaries (Hematoxylin & eosin, 400x original magnification).
Figure 6: Section of left lung demonstrating a pulmonary artery branch distended with thrombus and multiple thrombi in surrounding smaller vessels (Hematoxylin & eosin, 40x original magnification).
Figure 7: Hypopharyngeal mucosa and submucosal tissue demonstrating thrombi in multiple vessels (Hematoxylin & eosin, 20x original magnification).
Figure 8: Histological section of the neck skin demonstrating thrombi in deep dermal and subcutaneous vessels (Hematoxylin & eosin, 20x original magnification).