Abstract
Natural killer (NK)/T-cell lymphoma-associated hemophagocytic syndrome (HPS) is a rare and fatal disease with no optimal treatment. The present study reports the clinical features, diagnosis and treatment process of three patients with relapsed NK/T-cell lymphoma-associated HPS. All of the patients were classified as Ann Arbor stage IV and presented with a poor performance status. Two patients were successfully treated with a pegaspargase-containing combination regimen and one patient succumbed due to serious complications. These cases indicate that for patients with a history of lymphoma, the diagnosis of HPS should be considered when patients present with progressive high fever, pancytopenia and liver dysfunction. Early identification and effective treatments, including pegaspargase-based regimens are essential for an enhanced prognosis.
Highlights
Natural killer (NK)/T‐cell lymphoma is a heterogeneous and severe disease
hemophagocytic syndrome (HPS) is characterized by a high level of inflammatory cytokines secreted by activated T cells and aggressively proliferous macrophages
Secondary HPS is caused by underlying conditions, including infections, malignancies, autoimmune disorders and drugs, such as lamotrigine and etanercept, and is more prevalent in adults [4]
Summary
Natural killer (NK)/T‐cell lymphoma is a heterogeneous and severe disease. It is rare in Europe and North America, more common in Asia and South America. The current study presents three patients with relapsed NK/T‐cell lymphoma‐associated HPS.
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