Fatality in Kikuchi-Fujimoto disease: A rare phenomenon

Abstract

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.