Disseminated intravascular coagulopathy caused by Kikuchi–Fujimoto disease resulting in death: first case report in Turkey

Abstract

Kikuchi disease, also called Kikuchi–Fujimoto disease or Kikuchi’s histiocytic necrotizing lymphadenitis, is a rare, benign condition of unknown cause, usually characterized by cervical lymphadenopathy and fever. The diagnosis is based on histopathology. Our patient was a woman with bilateral cervical lymphadenopathy, fever, chest and abdominal pain, fatigue, maculopapular rash on her face, trunk, and upper and lower extremities. Immunological and rheumatological tests were negative. We took a cervical lymph node biopsy that showed a proliferative and necrotizing process centered in the paracortex characterized by patchy circumscribed or confluent areas of necrosis associated with karyorrhexis, and was remarkable by the absence of granulocytes and the paucity of plasma cells. These findings confirmed the diagnosis of Kikuchi’s disease. The patient’s hemoglobin values decreased, and the peripheral blood smear revealed schistocytes. Blood tests showed raised D-dimer, activated partial thromboplastin time, prothrombin time, and international normalized ratio with decreased fibrinogen. The patient’s condition quickly worsened and disseminated intravascular coagulopathy eventually developed. Her initial management consisted of a corticosteroid and hydroxychloroquine.

Recurrent Kikuchi's Disease Treated by Hydroxychloroquine

“…KFD patients can suffer classic symptoms, which include fever, fatigue, and cervical lymph node enlargement and can also have unusual presentation, such as axillary and inguinal lymph node enlargement, skin rash, arthralgia, splenomegaly, and aseptic meningitis [ 4 ]. KFD can even be complicated with fatal outcomes like disseminated intravascular coagulopathy (DIC), and pulmonary hemorrhage [ 14 ]. Patients with classic symptoms respond to NSAIDs or steroid, and those with severe symptoms usually respond to steroids.…”

“…Even studies from foreign countries are limited to case reports. In addition to the methods described above, there are case reports on the use of immunoglobulin, cyclosporine, minocycline, and ciprofloxacin for the treatment of patients with complicated KFD [ 6 14 ].…”

“…Considering that KFD was not associated with SLE, HC at 3 mg/kg/day was used for symptom control. There are several case reports of complicated KFD does not respond to NSAIDs and steroids [ 8 14 ]. Furthermore, the overall prevalence rate of KFD is increasing in Korea [ 3 ].…”

Evaluation of 44 Kikuchi-Fujimoto Disease Cases Reported from Turkey Between 2000-2010 with Pooled Analysis Method

“…11,18,30 Asya'da yaygın olarak görülen bu hastalık, başta Avrupa, Amerika Birleşik Devletleri ve Avustralya'da olmak üzere dünyanın birçok yerinden de bildirilmektedir. 23 Hastalığın ilk bildirilmeye baş-landığı dönemde olguların %80'ininden fazlasının kadın olduğu bildirilse de, son zamanlardaki serilerde bu oranın, 1.1:1 ile 2,75:1 kadın baskınlığı şeklinde olduğu görülmektedir. 19 Olgular genellikle 8-80 yaşları arasındadır; ortalama yaş 30 yıl-dır.…”

“…6,15,17,23,34 Lökopeni olguların %25-70'sinde görülürken, lökositoz olguların %5'inden daha azında izlenmektedir. 26,29,34 Bununla birlikte, olguların 1/3'ünde periferik kan yaymasında atipik lenfositler görülebilmektedir.…”