Table 1. Women meeting each of the HLH-2004 diagnostic criteria.
| Diagnostic criteria | Number of
women (total = 7) |
|---|---|
| Fever > 38.5 | 7 |
| Splenomegaly | 4 |
| Peripheral blood cytopaenias
(2/3 of: haemoglobin <90 g/L, platelets <100 x10 9/L, neutrophils <1 x10 9/L) |
6 |
| Hypertriglyceridaemia (>3 mmol/L) and/or hypofibrinogenemia
(<1.5 g/L) |
4 |
| Haemophagocystosis seen on biopsy of bone marrow/spleen/
lymph nodes |
7 |
| Low or absent natural killer (NK) cell activity | 1 |
| Ferritin >500 ng/ml | 5 |
| Soluble interleukin-2 receptor α (CD25) >2400 U/ml | 3 |