Difficulties in the diagnosis of histiocytic necrotizing lymphadenitis

Abstract

There is currently no consensus on the origin of histiocytic necrotising lymphadenitis (HNL), which is traditionally thought to be a self-limited, benign condition that usually resolves within 6 months. It is important to distinguish HNL as a clinical nosology because it can mimic other diseases such as lymphoma, infectious (mostly viral) and autoimmune diseases, including systemic lupus erythematosus. According to one study, HNL is misdiagnosed as lymphoma in 30% of cases. It has seen a similar clinical case in own practice.

Purpose - to highlight the thoroughness of the diagnostic algorithm and differential diagnosis in case of suspected HNL.

The article presents a clinical case of HNL in a 9-year-old child, which showed the complexity of clinical diagnosis. This observation combined the characteristic symptoms of the disease (fever, lymphadenopathy, hepatomegaly), haematological markers (leukemia, thrombocytopenia, anemia, accelerated erythrocyte sedimentation rate), as well as rare manifestations. There was a progressive development of edematous syndrome, which was manifested first by peripheral manifestations, and then bilateral pleurisy, ascites, soft tissue edema with the development of anasarca progressively increased.

The difficulty in the diagnostic algorithm was that the first two histological examinations suggested the possibility of lymphoma in the child, and later immunohistochemical examination of the lymph node allowed to verify the clinical diagnosis. Obviously, a labour-intensive differential diagnosis in HNL requires the exclusion of the subject range of possible diseases of infectious or autoimmune origin.

Conclusions. The diagnosis of HNL in the above observation was characterized by the complexity of the interpretation of clinical, morphological, histological studies, and only the result of immunohistochemical examination allowed to establish the diagnosis. In practice, the paediatrician should be properly aware of this pathology in order to refer the child to a paediatric hematologist in a timely manner. In the presence of a complex of clinical symptoms (prolonged fever, lymphadenopathy, rash, neurological symptoms), the possibility of a diagnosis of HNL should be considered.

The study was performed in accordance with the Declaration of Helsinki. The informed consent of the child's parents was obtained for the study.

The author declares no conflict of interest.