Histiocytic necrotizing lymphadenitis with systemic lupus erythematosus mimicking like lymphoma

Abstract

Histiocytic necrotizing lymphadenitis (HNL) is a rare idiopathic disorder. It is frequently underdiagnosed due to its nonspecific clinical features, mimicking like lymphomas, autoimmune diseases, and infectious reactive lymphadenopathy. Histological examination of the lymph node is the basis of diagnosis in HNL. We report a 40-year-old male patient who presented with fever for 4 weeks. After an extensive workup, laboratory investigations revealed progressive pancytopenia, gradual increase in serum lactate dehydrogenase, and ferritin with negative pyrexia profile. His radiological imaging including positron emission tomography/computed tomography showed multiple intra-abdominal, cervical, and axillary lymphadenopathies. Finally, the histological section from axillary lymph node core biopsy revealed a diagnosis of HNL. Subsequent autoimmune workup met diagnostic criteria for systemic lupus erythematosus. This case report alerts the clinicians regarding this rare disease and emphasizes the important role of morpho-histopathology in early recognition of this rare entity.

Keywords

• histiocytic necrotizing lymphadenitis
• histological examination
• pancytopenia
• systemic lupus erythematosus