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Table 1 Haemophagocytic Lymphohistiocytosis (HLH) 2004 clinical trial B diagnostic (clinical) criteria from 21 patients who had investigative work up for macrophage activation syndrome at presentation and underwent protein-based and degranulation screening tests for primary HLH from a single United Kingdom Paediatric Rheumatology centre

From: Screening assays for primary haemophagocytic lymphohistiocytosis in children presenting with suspected macrophage activation syndrome

 

Number of patients (%)

Number of patients with information available

HLH diagnosis: B Criteria

 Fever

19 (90)

21

 Splenomegaly

9 (47)

19

 Cytopenia (affected ≥2 of 3 lineages)

5 (24)

21

  Haemoglobin <9 g/dl

11 (52)

 

 (Infants <4 weeks: Haemoglobin <10 g/dl)

 

  Platelets <100 × 109/l

7 (33)

  Neutrophils <1 × 109/l

2 (1)

Hypertriglyceridaemia and/or hypofibrinogenaemia:

 Fasting triglycerides ≥3 mmol/l

8 (53)

15

 Fibrinogen ≤1.5 g/l

1 (0.1)

20

Haemophagocytosis in bone marrow or spleen or lymph nodes & no evidence of malignancy

7 (41)

17

Low or absent natural killer cell activity

Not tested

 

Ferritin ≥500 μg/l

14 (74)

19

aSoluble CD25 ≥2400 U/ml

4 (100)

4

  1. aOnly four patients had soluble CD25 levels tested. All were ≥2400 U/ml based on the original description of raised levels. It is acknowledged that >2SD from the mean is thought to be more meaningful
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Pediatric Rheumatology

ISSN: 1546-0096