A Diagnostic Dilemma: Similarity of Neuroradiological Findings in Central Nervous System Hemophagocytic Lymphohistiocytosis and Aspergillosis

Abstract

Central nervous system (CNS) involvement in the context of hemophagocytic lymphohistiocytosis (HLH) is not uncommon. Given the immunosuppressive nature of HLH therapy, infectious complications are also seen. We describe a 9-year-old male who developed acute neurological decline secondary to aspergillosis while undergoing HLH therapy. The significant overlap observed in CNS neuroimaging of HLH and aspergillosis and the subtleties that may help differentiate the two are discussed. The importance of obtaining tissue for definitive diagnosis is underscored.

Malignancy and chemotherapy induced haemophagocytic lymphohistiocytosis in children and adolescents—a single centre experience of 20 years

“…However, during treatment of malignancies, these two conditions might overlap and a clear etiologic discrimination might be difficult. Typical clinical symptoms of HLH may resemble symptoms of malignant diseases as well as severe bacterial infections often occurring during antineoplastic treatment and subsequent immunodeficiency [ 2 , 3 , 8 , 9 ]. Chemotherapy-associated HLH in fact is often reported to be triggered by infections and the hyperinflammatory state seems to be the consequence of an inadequate reaction of the dysregulated immune system against common infectious agents.…”

Central nervous system involvement in adult-onset hemophagocytic lymphohistiocytosis secondary to lymphoma: a case presentation and literature analysis

“…Of the 19 cases, deep white matter (14/19) was most frequently involved, followed by cortical or subcortical white matter regions (10/19), periventricular white matter (8/19), basal ganglia (8/19), and pons (5/19). Hemorrhage, perhaps due to perivascular infiltration causing injury, was reported in some cases (5/21) ( 7 , 9 , 25 , 26 ). Enhancement and diffusion restriction of the lesion was also reported in some cases.…”

Pulmonary Aspergillosis Complicated With Haemophagocytic Syndrome Secondary to Chronic Granulomatous Disease Description of the First Case

“…La aspergilosis pulmonar es una infección infrecuente en pacientes inmunocompetentes, siendo más frecuente en inmunocomprometidos [15][16] , mucho más aún lo es el síndrome hemofagocitico asociado a dicha infección 17 . Existen reportes muy escasos de asociación entre la presencia de aspergilosis pulmonar complicada con síndrome hemofagocitico, sobre todo en pacientes con condiciones de inmunosupresión como en el caso de neoplasias hematológicas [18][19] , en dichos casos la terapéutica está orientada a tratar la enfermedad de base [20][21] .…”