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Fellows’ Forum Case Report: Hemophagocytic Lymphohistiocytosis
The patient was a 48-year-old woman who saw her primary care physician for a flu-like illness three months prior to admission. Her symptoms initially improved, but recurred one month later; she was treated symptomatically, and again symptoms resolved. Two months later, she presented to an outside facility’s emergency department with fever to 103ºF, with associated…
Macrophage Activation Syndrome
A review of diagnosis, treatment, and prognosis
Diagnosing & Treating Hemophagocytic Lymphohistiocytosis in Adults
Primary hemophagocytic lymphohistiocytosis (HLH) is caused by genetic mutations and inherited syndromes; it therefore occurs in the pediatric age group. Secondary HLH, however, is more common in adults and is often triggered by other disease states, such as malignancies, chronic immunosuppression, infections and autoimmune disease.1,2 Macrophage activation syndrome (MAS) is a subset of secondary HLH…
Disease Mimicry: Genetic Diseases Can Produce Rheumatic Symptoms
CHICAGO—The 2018 ACR/ARHP Annual Meeting session on genetic mimics of rheumatic disease began with a description of a patient who presented with the symptoms of childhood-onset lupus but instead had a well-defined genetic disease: lysinuric protein intolerance (LPI). Lindsay Burrage, MD, PhD, assistant professor of molecular and human genetics at Baylor College of Medicine, Houston,…