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Special Feature
October 1998

Pathological Case of the Month

Arch Pediatr Adolesc Med. 1998;152(10):1033-1034. doi:10-1001/pubs.Pediatr Adolesc Med.-ISSN-1072-4710-152-10-ppc7045

A 5-YEAR-OLD boy with acute lymphoblastic leukemia presented with a 2-day history of fever and chills. The child had been treated with vincristine and L-asparaginase. He was receiving trimethoprim-sulfamethoxazole for Pneumocystis carinii prophylaxis, which was his only medication. Physical examination results were normal except for a mild left upper quadrant tenderness, but no organomegaly or masses.

Laboratory data included a hemoglobin level of 107 g/L, platelet count of 376×109/L, and white blood cell count of 10.3×109/L, with a normal differential cell count. Serum electrolytes, serum urea nitrogen, creatinine, serum transaminase, and amylase levels were all within normal limits. The chest radiograph revealed diffuse mild interstitial disease. Abdominal ultrasonography showed multiple small hypoechogenic foci within a normal-sized spleen and a normal-appearing liver, kidney, and pancreas. Abdominal computed tomography showed multiple small low-attenuation foci within the spleen, left lobe of the liver, and both kidneys (Figure 1).

The patient remained febrile despite treatment with cefuroxime and amphotericin. Daily cultures of blood and urine remained negative for organisms. On hospital day 5, the patient underwent splenectomy. Fever subsequently resolved and the patient was discharged 10 days postoperatively.

The resected spleen weighed 300 g. It had a bosselated surface caused by the presence of numerous subcapsular whitish nodules, better seen when the organ was serially cross-sectioned. This revealed a cut surface studded with discretely demarcated nodules measuring up to 0.7 cm in diameter (Figure 2). They were firm and whitish yellow with necrotic, cheesy-appearing centers. Each nodule was histologically a stellate-shaped granuloma made of epithelioid cells surrounding a central abscess, very reminiscent of the abscesses seen in cat-scratch disease (Figure 3). Staining for acid-fast bacilli, bacteria, and spirochetes (Warthin-Starry stain) remained negative, but the periodic acid–Schiff and the methenamine silver stains demonstrated the presence of aggregates of budding yeast and pseudohyphae in the center of the abscesses (Figure 4).