Hemophagocytic syndrome as the first presentation of histiocytic necrotizing lymphadenitis: report of 1 case and review of literature / 白血病·淋巴瘤

ABSTRACT

Objective:To investigate the diagnosis and treatment of histiocytic necrotizing lymphadenitis (HNL) complicated with hemophagocytic syndrome (HPS).Methods:The clinical characteristics, diagnosis, treatment process, and therapy response of a patient with HNL complicated with HPS admitted to the Institute of Hematology & Blood Diseases Hospital Chinese Academy of Medical Sciences in March 2019 were retrospectively analyzed, and the literature was reviewed.Results:This 17-year-old female patient had fever with bilateral cervical lymphadenopathy as the first presentation, accompanied by cough and expectoration. After admission, the disease progressed rapidly, and the serum ferritin increased progressively.The regimen of hormone and etoposide was used to control the disease condition. The bone marrow smear revealed atypical lymphocytes and hemophagocytic phenomenon, and the pathological features of HNL in lymph node biopsy were observed. This patient was finally diagnosed as HNL complicated with HPS. The patient's condition was stable at 3-month follow-up after discharge.Conclusions:The clinical manifestations of HNL patients complicated with HPS are similar to other hematologic malignant diseases, and application of multiple laboratory and pathological examination methods can help with early diagnosis. In the event of a progressive rise in serum ferritin, timely application of hormone therapy combined with etoposide if necessary can rapidly control the progression of the disease.