Case Report
Copyright ©The Author(s) 2017. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Feb 16, 2017; 5(2): 35-39
Published online Feb 16, 2017. doi: 10.12998/wjcc.v5.i2.35
Fatality in Kikuchi-Fujimoto disease: A rare phenomenon
Bianca Barbat, Ruby Jhaj, Daniyeh Khurram
Bianca Barbat, Ruby Jhaj, Daniyeh Khurram, Providence-Providence Park Hospital, Southfield, MI 48075, United States
Author contributions: All authors contributed to the acquisition of data, writing, and revision of this manuscript.
Institutional review board statement: This case report was exempted from the IRB standards at St. John Providence Hospital.
Informed consent statement: Verbal consent was granted by the patient’s family.
Conflict-of-interest statement: All authors have no conflict of interest to declare.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Correspondence to: Bianca Barbat, MD, Providence-Providence Park Hospital, 16001 W. 9 Mile Rd, Southfield, MI 48075, United States.
Telephone: +1-248-8493150
Received: July 1, 2016
Peer-review started: July 3, 2016
First decision: September 5, 2016
Revised: November 19, 2016
Accepted: December 27, 2016
Article in press: December 28, 2016
Published online: February 16, 2017

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is an uncommon condition, typically characterized by lymphadenopathy and fevers. It usually has a benign course; however, it may progress to fatality in extremely rare occasions. The diagnosis is made via lymph node biopsy and histopathology. Our patient was a young female who presented with shortness of breath, fever, and malaise. Physical examination revealed significant cervical and axillary lymphadenopathy. Chest X-ray displayed multilobar pneumonia. She required intubation and mechanical ventilation for progressive respiratory distress. Histopathology of lymph nodes demonstrated variable involvement of patchy areas of necrosis within the paracortex composed of karyorrhectic debris with abundant histiocytes consistent with KFD. After initial stabilization, the patient’s condition quickly deteriorated with acute anemia, thrombocytopenia and elevated prothrombin time, partial prothrombin time, and D-dimer levels. Disseminated intravascular coagulopathy (DIC) ensued resulting in the patient’s fatality. DIC in KFD is not well understood, but it is an important cause of mortality in patients with aggressive disease.

Keywords: Kikuchi-Fujimoto disease, Disseminated intravascular coagulopathy, Histiocytic necrotizing lymphadenitis, Lymphadenopathy, Fatality

Core tip: Kikuchi-Fujimoto disease (KFD), or histiocytic necrotizing lymphadenitis, is an uncommon condition, typically characterized by lymphadenopathy and fevers. KFD is an extremely rare disease. With this case we wish to highlight that KFD carries a risk of mortality in the setting of acute, aggressive disease, which is in contrast to the benign, self-limiting condition that has been classically documented in literature. The patient emphasizes the importance of recognizing this as we present the fourth case of disseminated intravascular coagulopathy as a cause of fatality in these patients.