AI Article Synopsis
- Hemophagocytic lymphohistiocytosis (HLH) is an immune system disorder that can be either hereditary (primary HLH) or caused by external factors (secondary HLH).
- The standard HLH-2004 criteria for diagnosis may lack specificity, often resulting in misdiagnosis and inappropriate treatment, especially in cases involving underlying cancers.
- An alternative diagnostic method, utilizing flow cytometry and a detailed look for malignancies, can provide more accurate results and expedite appropriate treatment without waiting for genetic tests.
Article Abstract
Hemophagocytic lymphohistiocytosis (HLH) is characterized by dysregulated immune activation. Primary HLH involves hereditary deficits in cytotoxic lymphocytes while secondary HLH is triggered by extrinsic factors. The HLH-2004 criteria are widely used for clinical diagnosis, yet their specificity for HLH or their ability to differentiate primary from secondary disease is unclear, potentially leading to inappropriate treatment. We describe several cases where fulfillment of HLH-2004 criteria obscured the diagnoses of underlying malignancies and delayed curative management. These issues are remedied without waiting for genetic testing results through an alternative diagnostic approach using flow cytometry-based immunologic assays and a thorough investigation for malignancy.
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| http://dx.doi.org/10.1002/pbc.27400 | DOI Listing |
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