Splendore-Hoeppli Phenomenon

Abstract

The patient was a 61-year-old woman with a 10-year history of chronic lymphocytic leukemia. Although she initially refused treatment, she received prednisone following the development of autoimmune hemolytic anemia. During the previous 3 years, her peripheral white blood cell count had remained stable, but she had been subject to numerous infections including Legionnaire's disease, herpes simplex lymphadenitis, and Clostridium difficile colitis. One week prior to admission, the patient developed severe right flank pain. A computed tomographic scan revealed a suprarenal mycotic aneurysm of the aorta. The patient underwent surgical repair of the aneurysm followed by splenectomy.Histologic examination of the aortic aneurysm was unrevealing. The excised spleen weighed 667 g and, upon sectioning, showed multiple, small (0.3–0.5 cm), white, intraparenchymal lesions. Histologic examination showed expansion of the red pulp and effacement of the white pulp by chronic lymphocytic leukemia and, in addition, several abscesses containing brightly eosinophilic crystalline structures in the “starburst” pattern of Splendore-Hoeppli material (Figure, A and B). Gram stain (Figure, C) and a modified acid-fast stain (Figure, D) revealed gram-positive, partially acid-fast filamentous organisms branching at right angles within the eosinophilic material, consistent with Nocardia organisms. Culture of the aortic aneurysm and the spleen confirmed infection by Nocardia asteroides.Chronic lymphocytic leukemia can result in marked immune dysfunction and immunodeficiency.1 Sequelae include hemolytic anemia and, in combination with steroid use, infections by opportunistic organisms.2The Splendore-Hoeppli phenomenon describes the eosinophilic, pseudomycotic structures composed of necrotic debris and immunoglobulin. In the United States, it is most frequently found in association with botryomycotic infections caused by Staphylococcus aureus and Pseudomonas aeruginosa. In the tropics, it can be found surrounding schistosome eggs, microfilariae, and a variety of fungi.3Nocardiae are opportunistic bacteria that most frequently cause pneumonia in immunosuppressed hosts. Systemic dissemination occurs in about one third of infected immunocompromised patients and usually elicits an intense inflammatory reaction resulting in necrotic abscesses. Unlike Actinomyces organisms, nocardiae are weakly acid fast. Dense colony formation by nocardiae, as seen in this case, is uncommon.4