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.

Evaluation of macrophage activation syndrome in hospitalised patients with Kikuchi-Fujimoto disease based on the 2016 EULAR/ACR/PRINTO classification criteria

“…Therefore, both KFD and HLH are associated with abnormal histiocyte activation. In addition, inflammatory cytokines such as tumor necrosis factor (TNF)-α, interleukin (IL)-1, and IL-6 are also implicated in the pathogenesis of both MAS and severe cases of KFD [30, 31]. Therefore, although it is possible that HLH can be accompanied by KFD, the association between KFD and HLH is not well understood.…”

“…Third, data regarding pro-inflammatory cytokines (TNF-α, interferon-γ, IL-1, IL-6, and soluble CD163) associated with MAS were not obtainable because the following tests were not available in our hospitals [36, 37]. Because these cytokines are known to play an important role in the pathogenesis of MAS [30, 31], assessment of such tests in future studies would provide additional significance. Fourth, because the 2016 classification criteria for MAS have only been validated in patients with sJIA, caution should be made when applying the criteria to non-sJIA diseases.…”

Kikuchi-Fujimoto Disease in Michigan: A Rare Case Report and Review of the Literature

“…Despite aggressive Intravenous (IV) antibiotics and high-dose steroids, this patient’s clinical course declined, eventually passing away due to the development of disseminated intravascular coagulation (DIC). 24 In addition to the case report described above, there have been 3 other case reports published which cite DIC as the cause of death in patients also diagnosed with KFD. 25,26 The association between DIC and KFD is not yet understood, and little is known how or if at all KFD plays a role in the development of DIC.…”

“…25,26 The association between DIC and KFD is not yet understood, and little is known how or if at all KFD plays a role in the development of DIC. 24 Apart from DIC as the cause of mortality, there have been other causes of death reported in patients with KFD, which include hemophagocytic syndrome and severe infection, 27 pulmonary hemorrhage, 28 and acute heart failure. 29 It is imperative to understand that these case reports do not imply a causal relationship between KFD and the development of DIC or other life-threatening conditions mentioned above, but rather an association.…”

Case Report of Kikuchi-Fujimoto Disease from Sub-Saharan Africa: An Important Mimic of Tuberculous Lymphadenitis

“…Irrespective of the form of intervention, the prognosis of the condition is reported as favorable. However, there have been reported cases of death especially in patients who develop DIC [29].…”