Leukocytosis and high hematocrit levels during abdominal attacks of hereditary angioedema

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• 作者：Isao Ohsawa (11)
  Seiji Nagamachi (11)
  Hiyori Suzuki (11)
  Daisuke Honda (11)
  Nobuyuki Sato (11)
  Hiroyuki Ohi (12)
  Satoshi Horikoshi (11)
  Yasuhiko Tomino (11)
  
• 关键词：Hereditary angioedema ; C1 ; inhibitor ; C1 ; inhibitor concentrate ; Acute abdomen ; Leukocytosis ; Hemoconcentration
• 刊名：BMC Gastroenterology
• 出版年：2013
• 出版时间：December 2013
• 年：2013
• 卷：13
• 期：1
• 全文大小：248KB
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  22. The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-230X/13/123/prepub
  
• 作者单位：Isao Ohsawa (11)
  Seiji Nagamachi (11)
  Hiyori Suzuki (11)
  Daisuke Honda (11)
  Nobuyuki Sato (11)
  Hiroyuki Ohi (12)
  Satoshi Horikoshi (11)
  Yasuhiko Tomino (11)
  
  11. Division of Nephrology, Department of Internal Medicine, Juntendo University Faculty of Medicine, Tokyo, Japan
  12. Internal Medicine, Tsurumi-Nishiguchi Hospital, Kanagawa, Japan
  

文摘

Background The diagnosis of hereditary angioedema (HAE) is often delayed due to the low awareness of this condition. In patients with undiagnosed HAE, abdominal symptoms often create the risk of unnecessary surgical operation and/or drug therapy. To explore the cause of misdiagnosis, we compared the laboratory findings of HAE patients under normal conditions with those during abdominal attacks. Methods Patient medical histories were analyzed and laboratory data at the first consultation with no symptoms and no medication were compared with those at visits to the emergency department during severe attacks. Results Fourteen HAE patients were enrolled. Initial HAE symptoms occurred at 20.2 ± 9.4 years of age. The correct diagnosis of HAE was made 22.7 ± 14.2 years after the initial symptoms. A common site of angioedema was the extremities. Half of the patients experienced a life-threatening laryngeal attack and/or severe abdominal pain. In the patients with severe abdominal pain, significant leukocytosis with neutrophilia along with increased levels of hematocrit were observed while levels of C-reactive protein (CRP) remained low. All severe attacks were alleviated with an infusion of C1-inhibitor concentrate. Conclusions Consideration of the likelihood of a HAE attack is important when patients present with acute abdominal pain and leukocytosis without elevation of CRP.