- 期刊
週期性嗜中性白血球減少症－父子三病例報告－
Cyclic Neutropenia: Report of Three Cases in a Family
摘要
週期性嗜中性白血球減少症之原因不明，其周邊血液血球數會出現週期性變化，有很強的家族傾向，患者可反覆出現發燒、口內潰瘍、或皮膚感染等症狀，但很少同為嚴重感染而致命。本文報告之家族中，有一個9歲男童，因為上述症狀而接受一系列檢查，發現其週邊血液嗜中性白血球及單核球數，呈現20至24天的週期性升降變化，其他實驗室檢查及身體檢查均正常。對其他家庭成員的研究中發現，其弟弟也有18至22天的週期性血球數變化，共父親則呈持續嗜中性白血球減少，我們皆診斷之為週期性嗜中性白血球減少症。兄弟兩病童曾接受prednisolone (2 mg/kg/day)治療，後因體重和食慾明顯增加，而自動停藥。追踪至今的兩年間，病人雖仍有反覆成染現象，但生長髮育正常。
關鍵字
無資料並列摘要
Cyclic neutropenia is characterized by cyclic disappearance of neutrophils from the circulation, with a typical periodicity of 20-21 days. The disease is transmitted as an autosomal dominant of high penetrance. Fever, oral ulceration and skin infections are the predominant features and it usually runs a benign course, although death from infection has occurred in a few cases. This is a presentation of three cases of cyclic neutropenia from a single family. The proband is a 9-year-old boy who has suffered from frequent fever, oral ulceration and sore throat since early childhood. Serial peripheral hemograms showed cyclic oscillation of neutrophils, monocytes and reticulocytes with a periodicity of 20-24 days. Bone marrow examination during nadir interval revealed mild hypocellularity and poor maturation sequence of myeloid series. Family study showed that his younger brother also has the same problem with a periadicity of 18-22 days. The father has persistent neutropenia, which is also regarded as a case of cyclic neutropenia.