Table 1.
Baseline characteristics and hemophagocytic lymphohistiocytosis diagnosis of patient
|
Variables
|
Patient
|
| Baseline characteristics | |
| Age (yr) | 36 |
| Etiology/Trigger | SLE and multiple infections |
| HLH- directed therapies | VP16 and methylprednisolone |
| HLH- 2004 criteria at diagnosis (ref.: Henter-2007) | |
| Fever | Y |
| Splenomegaly | N |
| Cytopenia, affecting 2 of 3 lineages in the peripheral blood | |
| Hemoglobin concentration < 9 g/dL | Y |
| Neutrophil count < 1.0 × 109/L | Y |
| Platelet count < 100 × 109/L | Y |
| Hypertriglyceridemia (fasting ≥ 3.0 mmol/L) and/or hypofibrinogenemia (≤ 150 mg/dL) | Y |
| Hemophagocytosis in bone marrow or spleen or lymph nodes (no evidence of malignancy) | N |
| Low or absent natural killer cell activity | N/A |
| Ferritin ≥ 500 ng/mL | Y |
| Soluble cluster of differentiation 25 (i.e. soluble interleukin 2 receptor) ≥ 2400 U/mL | N |
HLH: Hemophagocytic lymphohistiocytosis; N/A: Not applicable; Y: Yes; N: No; ref.: References.