Case 4--Multiple Nodules with Crystals

Clinical summary: A 77-year-old man with a 20-year history of neutropenia was admitted to the hospital for investigation of jaundice of 3 days' duration. The neutropenia was accompanied by an ANA positive at 1:80 to 1:160. Total WBCs varied from 1.1 to 1.4 k/µl with 800 granulocytes/µl. He had mild anemia (Hb 12.2-13.2 g/dl) and a normal platelet count (211 k/µl). The peripheral smear showed no blasts or promyelocytes. A chest radiograph 3 months before admission was stated to be normal. On admission, the serum bilirubin was 13 mg/dl. Endoscopic retrograde cholangiopancreatography and CT were consistent with carcinoma of the pancreas without local spread, and he underwent a Whipple procedure. Postoperatively, he developed methicillin-resistant S. aureus septicemia and died on the 16th postoperative day.

At autopsy the lungs were congested and had acute bronchopneumonia. An incidental finding is shown here. Scattered throughout all lobes were cavitated lesions up to 1 cm in diameter with firm, yellowish nodules (arrow).

A section from the nodular portion of the lesion shows an abrupt transition from almost normal alveolar parenchyma (right) to the lesion. There is destruction of the normal alveolar architecture leaving a cavity with nodules.

The lesion (left) has central homogeneous, pink material surrounded by fibrosis. The surrounding alveolar parenchyma has a mild lymphoid infiltrate. What special stain would help to identify the central pink material? Answer

Clinical summary Case 4 continued

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The material appeared to be amyloid, but a Congo red stain showed neither red staining nor green birefringence with polarized light.

Electron microscopy

At low-power, electron microscopy showed material of different densities. At high-power, there is a diffuse, fine granularity but no fibrils of amyloid. What is this material? Answer

Low-power view

High-power view

Clinical summary Case 4 continued

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Answer: The material is consistent with non-amyloid light chain deposition. What special stain would support this diagnosis? Answer

Low-power view

High-power view

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Answer: A positive PAS-D stain and demonstration of a clonal cellular proliferation would confirm the diagnosis.

Clinical summary Case 4 continued

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