P athology I mage of the M onth Death in a Young Adult With Sickle Cell Disease
Joel France, DO; Robin R. McGoey, MD
A 27-year-old African-American female with known sickle cell disease was admitted for sickle cell crisis and presumed sepsis. The patient’s past medical history was complicated by multiple sickle cell-related complications, including seizures and multiple prior blood transfusions. Her hospital course included Staphylococcus epidermidis bacteremia, for which broad spectrum antibiotics were prescribed. On hospital day nine, the patient was found unresponsive and declared dead after unsuccessful efforts at resuscitation. An unlimited autopsy examination was conducted under authorization of the coroner. Findings included numerous pathologic features ascribed to sickle cell disease, including systemic siderosis and splenic at- rophy [weight 10gm (140±78)], fibrosis, and Gamna Gandy nodules. Additional autopsy findings included cardiomegaly with a heart weight of 450gm (312±78), right atrial and right ventricular chamber dilatation, and hepatomegaly with a liver weight of 2650gm (1475±362). The image below demonstrates microscopic examination of the lung parenchyma.
Figure 1: Histologic section from lung parenchyma demonstrating a small caliber pulmonary artery with an intraluminal plexus of capillary-like channels separated by proliferating intimal cells and resulting in luminal stenosis and superimposed thrombotic material (40x, Hematoxylin and Eosin stain).
What is the name of this hallmark arterial lesion? What clinical disorder does it represent?
J La State Med Soc VOL 166 September/October 2014 231
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