A 32-year-old woman was admitted to hospital complaining of fever, fatigue, as well as chest and abdominal pain of 15 days' duration, and with no personal and family history for tuberculosis and malignant diseases. Vital signs in the emergency department revealed a temperature of 39 C, a blood pressure level of 140/80 mmHg, a heart rate of 88 bpm, and a respiratory rate of 16 breaths/minute. On physical examination, she was lethargic and maculopapular rashes were patent on her face, trunk, and upper and lower extremities. She had bilateral enlarged cervical, axillary, and inguinal lymph nodes, which were freely mobile, painless on palpation, discrete and sized in the range of 0.5-3.7 cm. In Figure 1, computed tomography of the neck showed the presence of lymphadenomegalies of various sizes. Cardiovascular and respiratory examinations were unremarkable. Initial laboratory studies revealed hemoglobin levels of 10.2 g/dL, leukocytes of 17,800/mm3 with an abnormal differential count of eosinophils of 9.8%, neutrophils of 54.3%, monocytes of 14%, platelets of 205,000/mm3, lactate dehydrogenase of 578 U/L, C-reactive protein of 28.2 mg/L, and an erythrocyte sedimentation rate (ESR) of 77 mm/hour. Liver and renal tests were normal. Serologic tests for toxoplasma, Epstein-Barr virus, rubella, cytomegalovirus, herpes simplex type 1, hepatitis B, and hepatitis C were all negative. Blood, urine, and sputum cultures were negative. Sputum culture was also negative for acid-fast bacilli. Protein electrophoresis showed polyclonal gammopathy. Also, immunological and rheumatological tests, including antinuclear antibody, deoxyribonucleic acid antibody, perinuclear anti-neutrophil cytoplasmic antibodies, antineutrophil cytoplasmic antibodies, extractable nuclear antigens, and rheumatoid factor were all negative. Electrocardiogram and chest radiographs were normal.
Computed tomography demonstrated generalized lymphadenopathy in the axillae, cervical area, paraaortic areas, inguinal area, and mediastinum.
We took a cervical lymph node biopsy that showed karyorrhectic debris and the cellular infiltration of histiocytes and immunoblasts in the absence of polymorphonuclear leukocytes (Figure 2) and activated B-cells with histiocytes (Figure 3). An immunohistochemical study showed that the lymphoid cells adjacent to the necrotic foci were strongly positive for cluster of differentiation (CD)3, and the histiocytic cells at the periphery of the necrotic foci were strongly positive for CD68, so we thought that this histological pattern was suggestive of KFD.
After diagnosis, the patient received corticosteroid and hydroxychloroquine therapy. During treatment, the patient's clinical and laboratory parameters worsened. The patient's diagnosis was revised, but new findings were unremarkable for a particular 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 eventually disseminated intravascular coagulopathy (DIC) developed. She remained completely unresponsive to therapy and she subsequently died.