Macrophage Activation Syndrome Revealing Hodgkin Lymphoma: A Pediatric Case Report

Abstract

Secondary macrophage activation syndrome is a severe and rare complication of infectious, autoimmune, and malignant diseases. Herein, we report the case of a 10-year-old child with this syndrome leading to the discovery of Hodgkin lymphoma. The patient, who had a medical history of cerebral palsy and ataxia-telangiectasia syndrome, was admitted for prolonged fever associated with cough, which had evolved into general health deterioration for two months prior to admission. He received multiple courses of antibiotic therapy for suspected pneumonia without clinical improvement. Clinical examination showed pallor, deteriorated general condition, high fever, asthenia, cervical adenopathy, and splenomegaly. The clinical and biological criteria led to the diagnosis of macrophage activation syndrome. Lymph node histology confirmed the diagnosis of Hodgkin lymphoma, which was staged as stage IV via positron emission tomography scan. The "vincristine, etoposide, prednisone, and doxorubicin" (OEPA) chemotherapy regimen was initiated, followed by the "cyclophosphamide, vincristine, prednisone, and dacarbazine" (COPDAC) regimen, with remission achieved both clinically and paraclinically after one cycle of each regimen.

Keywords: adenopathy; fever; histology; hodgkin lymphoma; macrophage activation syndrome.

Figure 1. PET-CT axial views of hypermetabolic lesions on prevascular node (A), spleen (B) and liver (C) nodes, as well as a pulmonary nodule (D).