• P G Scimeca, A G James-Herry, and M E Weinblatt.
• Division of Pediatric Hematology/Oncology, North Shore University Hospital-Cornell University Medical College, Manhasset, New York 11030, USA.
• J. Pediatr. Hematol. Oncol. 1996 May 1; 18 (2): 159-61.

PurposeTo describe a case of atypical, severe, periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (PFAPA syndrome) in a patient with Fanconi anemia. Important aspects about the PFAPA syndrome and Fanconi anemia are reviewed.Patients And MethodsAn 8-year-old girl with Fanconi anemia was noted to have a pattern of periodic fever, stomatitis, and pharyngitis consistent with the diagnosis of PFAPA syndrome, a generally benign disorder. After prednisone treatment for the syndrome, life-threatening intestinal ulceration and perforation developed, which was successfully treated.ConclusionIn patients with underlying hematologic disease such as Fanconi anemia, PFAPA syndrome may be associated with severe clinical problems in contrast to otherwise normal children with the disorder.

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