Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access
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Case Report

Volume 000, Number 000, February 2025, pages 000-000


Hemophagocytic Lymphohistiocytosis After Treatment With Checkpoint Inhibitor Therapy

Table

Table 1. HLH-2004 Diagnostic Criteria
 
The diagnosis of HLH can be established if criterion 1 or 2 is fulfilled. HLH: hemophagocytic lymphohistiocytosis; NK: natural killer; sCD25: soluble CD25; IL-2: interleukin-2.
Criterion 1. A molecular diagnosis consistent with HLH
Criterion 2. Diagnostic criteria for HLH fulfilled (five of the eight criteria below)
  1) Fever
  2) Splenomegaly
  3) Cytopenias (affecting ≥ 2 of 3 lineages in peripheral blood)
  4) Hemoglobin < 90 g/L (hemoglobin < 100 g/L in infants < 4 weeks)
  5) Platelets < 100 × 109/L
  6) Neutrophils < 1.0 × 109/L
  7) Hypertriglyceridemia and/or hypofibrinogenemia
  8) Fasting triglycerides > 3.0 mmol/L (i.e., > 265 mg/dL)
  9) Fibrinogen ≤ 1.5 g/L
  10) Hemophagocytosis in the bone marrow, spleen or lymph node
  11) No evidence of malignancy
  12) Low or no NK cell activity (according to local laboratory reference)
  13) Ferritin ≥ 500 µg/L
  14) sCD25 (i.e., soluble IL-2 receptor) ≥ 2,400 U/mL