Fever of Multiple Possible Origins Acquired Hemophagocytic Lymphohistiocytosis in the Context of Pyelonephritis, Newly Diagnosed Systemic Lupus Erythematosus, and an Ulcer of the Aortic Arch

Main Article Content

David Spillane
Jeffrey Wiseman

Acquired Hemophagocytic Lymphohistiocytosis , Pyelonephritis, lupus

Abstract

A 60-year-old man presented with 1 week of fever despite broad-spectrum antibiotics for presumed pyelonephritis based on extended spectrum bacteriuria, recent bladder catheterization, and a negative search for other infections. He developed a maculopapular truncal rash, and pancytopenia with persistent fevers and worsening inflammatory markers despite modifying then stopping antibiotics. The non-specific clinical features at presentation and absence of hemophagocytosis on the initial bone marrow aspirate confounded multiple subspecialists and delayed the final diagnosis of hemophagocytic lymphohistiocytosis (HLH). Once this syndrome was elucidated, he responded well to dexamethasone and etoposide. An underlying diagnosis of systemic lupus erythematosus with aortic vasculitis was made, which in combination with pyelonephritis likely precipitated HLH. We summarize current concepts, pitfalls, and lessons learned in the diagnosis and management of HLH.



Résumé
Un homme de 60 ans se présente à l’hôpital à la suite d’une semaine de fièvre malgré la prise d’antibiotiques à large spectre pour traiter une pyélonéphrite soupçonnée, fondée sur une bactériurie à spectre étendu, un cathétérisme vésical récent et une recherche infructueuse d’autres infections. Il a développé une éruption cutanée maculopapulaire sur le tronc et une pancytopénie accompagnée d’une fièvre persistante et d’une augmentation des marqueurs de l’inflammation malgré la modification, puis l’arrêt des antibiotiques. Les manifestations cliniques non spécifiques à la présentation et l’absence d’hémophagocytose lors de la ponction médullaire initiale ont confondu de multiples surspécialistes et retardé le diagnostic définitif de lymphohistiocytose hémophagocytaire (LHH). Une fois que ce syndrome a été élucidé, le patient a bien répondu au traitement par la dexaméthasone et l’étoposide. Un diagnostic sous-jacent de lupus érythémateux systémique accompagné d’une vascularite de l’aorte a été posé qui, combiné à la pyélonéphrite, a probablement précipité la LHH. Nous résumons les concepts actuels, les pièges et les leçons apprises dans le diagnostic et la prise en charge de la LHH.

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