A case of cirrhosis concurrent with NK/T-Cell lymphoma and hemophagocytic lymphohistiocytosis: a clinical report and literature review.

in BMC infectious diseases by Delei Li, Xu Xiang

TLDR

  • A rare case of cirrhosis concurrent with NK/T-cell lymphoma and hemophagocytic lymphohistiocytosis (HLH) highlights the importance of early recognition and coordinated multidisciplinary interventions in optimizing outcomes.
  • Laboratory findings, including elevated ferritin and hypercytokinemia, and histopathological examination confirmed the diagnoses of NK/T-cell lymphoma and HLH.
  • The case emphasizes the need for a multidisciplinary approach in managing patients with this rare and complex disease.

Abstract

This study aimed to investigate the clinical manifestations and therapeutic strategies for cirrhosis concurrent with NK/T-cell lymphoma and hemophagocytic lymphohistiocytosis (HLH), to enhance diagnostic and therapeutic insights into this rare disease with triple comorbidity. We retrospectively analyzed the clinical data and diagnostic-therapeutic workflow of a patient admitted to our hospital in August 2024 with cirrhosis, NK/T-cell lymphoma, and HLH. The patient was admitted to the Department of Infectious Diseases of our hospital with cirrhosis of unknown origin. Laboratory findings demonstrated trilineage cytopenia, coagulopathy, deranged blood biochemistry, markedly elevated ferritin, and hypercytokinemia. Serological testing confirmed Epstein-Barr virus (EBV) antibody positivity, while molecular assays detected high EBV DNA loads. Bone marrow cytomorphology and cervical lymph node histopathology corroborated NK/T-cell lymphoma with HLH. Whole-body PET/CT revealed multifocal lymph node involvement. Following multidisciplinary evaluation, the patient underwent hematology-directed management combining hepatoprotective therapy, antimicrobials, and chemotherapy (gemcitabine/oxaliplatin/mitoxantrone liposome), stabilizing disease. The co-occurrence of cirrhosis, NK/T-cell lymphoma, and HLH represents a rare clinical entity with a grim prognosis. This case highlights the necessity of early recognition and coordinated multidisciplinary interventions to optimize outcomes.

Overview

  • The study aimed to investigate the clinical manifestations and therapeutic strategies for cirrhosis concurrent with NK/T-cell lymphoma and hemophagocytic lymphohistiocytosis (HLH) in a rare triple comorbidity disease.
  • The case study involved a patient with unknown-origin cirrhosis, who was later diagnosed with NK/T-cell lymphoma and HLH, and underwent hematology-directed management combining hepatoprotective therapy, antimicrobials, and chemotherapy.
  • The study highlights the importance of early recognition and coordinated multidisciplinary interventions to optimize outcomes in patients with this rare and complex disease.

Comparative Analysis & Findings

  • Laboratory findings revealed trilineage cytopenia, coagulopathy, deranged blood biochemistry, markedly elevated ferritin, and hypercytokinemia.
  • Serological testing confirmed Epstein-Barr virus (EBV) antibody positivity, while molecular assays detected high EBV DNA loads.
  • Bone marrow cytomorphology and cervical lymph node histopathology corroborated NK/T-cell lymphoma with HLH, and whole-body PET/CT revealed multifocal lymph node involvement.

Implications and Future Directions

  • This case highlights the importance of a multidisciplinary approach in managing patients with this rare disease, involving hematology, infectious disease, and liver specialists.
  • Further research is needed to better understand the pathophysiology of this triple comorbidity disease and to develop effective therapeutic strategies.
  • The study suggests that early recognition and coordinated management may improve outcomes in patients with this disease, and highlights the need for increased awareness among healthcare providers.
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