Scimeca P G, James-Herry A G, Weinblatt M E
Division of Pediatric Hematology/Oncology, North Shore University Hospital-Cornell University Medical College, Manhasset, New York 11030, USA.
J Pediatr Hematol Oncol. 1996 May;18(2):159-61. doi: 10.1097/00043426-199605000-00012.
To 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.
An 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.
In 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.
描述1例患有范可尼贫血的患者出现非典型、严重、周期性发热、口疮性口炎、咽炎和腺炎综合征（PFAPA综合征）的病例。对PFAPA综合征和范可尼贫血的重要方面进行综述。
一名8岁患有范可尼贫血的女孩被发现有周期性发热、口炎和咽炎模式，符合PFAPA综合征的诊断，这是一种通常为良性的疾病。在对该综合征进行泼尼松治疗后，出现了危及生命的肠道溃疡和穿孔，并成功得到治疗。
与患有该疾病的正常儿童相比，患有潜在血液系统疾病如范可尼贫血的患者，PFAPA综合征可能与严重的临床问题相关。
