Abstract
BackgroundAdult hemophagocytic lymphohistiocytosis is a secondary immunopathologic phenomenon, mainly secondary to malignancy, infection, or autoimmune disorders.The performance of diagnostic criteria, studied in the pediatric population, is yet to be validated in the adult population. Some of the criteria include cytopenias and organomegaly that are inherent features to malignant processes, thus making the diagnosis of hemophagocytic lymphohistiocytosis a challenge in patients with cancer.Case presentationWe describe the case of a 54-year-old white man with history of metastatic maxillary sinus adenoid cystic carcinoma who had severe liver injury and cytopenias with progressive clinical deterioration. We performed an evaluation, by flow cytometry, of the expression of surface markers in his natural killer cells that revealed remarkable abnormalities. His syndrome eventually fulfilled criteria for hemophagocytic lymphohistiocytosis and he received therapy with steroids with interval clinical improvement. Unfortunately, he refused further cytotoxic treatment and died 2 weeks later.ConclusionsThe conventional criteria for the diagnosis of hemophagocytic lymphohistiocytosis are suboptimal for adult patients with cancer resulting in delays in diagnosis and timely initiation of treatment. The diagnostic criteria have to be re-evaluated in patients with cancer; novel, easily available, and accurate diagnostic methods are needed.
Highlights
Adult hemophagocytic lymphohistiocytosis is a secondary immunopathologic phenomenon, mainly secondary to malignancy, infection, or autoimmune disorders
The conventional criteria for the diagnosis of hemophagocytic lymphohistiocytosis are suboptimal for adult patients with cancer resulting in delays in diagnosis and timely initiation of treatment
The diagnostic criteria have to be reevaluated in patients with cancer; novel, available, and accurate diagnostic methods are needed
Summary
The current diagnostic criteria of HLH might be suboptimal for adult patients and perhaps result in delays in its diagnosis and timely initiation of treatment. BRB conceived the idea for the manuscript, contributed to drafting of the manuscript and final approval of the version to be published. ND conceived the idea for the manuscript, contributed to drafting of the manuscript and final approval of the version to be published. CMR was the lead physician responsible for the clinical care of the case, supplied all clinical details, drafting of the manuscript, and final approval of the version to be published. Author details 1Department of Internal Medicine, The University of Texas Health Science Center, Houston, TX, USA.
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