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2017 ; 5
(2
): 35-39
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Fatality in Kikuchi-Fujimoto disease: A rare phenomenon
#MMPMID28255545
Barbat B
; Jhaj R
; Khurram D
World J Clin Cases
2017[Feb]; 5
(2
): 35-39
PMID28255545
show ga
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.