Table 1 Clinical features and laboratory findings of each of the six patients with adult‐onset Still's disease and reactive haemophagocytic syndrome.
| Case no 1 | Case no 2 | Case no 3 | Case no 4 | Case no 5 | Case no 6 | |
| Sex/age at onset of RHS | F/38 y | F/22 y | M/44.5 y | M/72 y | F/47 y | F/36 y |
| AOSD duration before RHS | 2 y | 0 | 7.5 y | 4.5 y | 1 mo | 1 mo |
| Previous IS therapy | CS | − | CS | − | − | − |
| Clinical findings | ||||||
| Fever >39°C | + | + | + | + | + | + |
| Rash | − | + | + | − | + | − |
| Arthralgia | − | + | + | − | − | + |
| Myalgia | − | + | + | − | + | − |
| Sore throat | − | + | − | + | + | − |
| Lymphadenopathy | − | + | − | − | − | + |
| Splenomegaly | + | − | − | − | − | + |
| Hepatomegaly | − | − | − | − | − | + |
| Pleuritis | − | − | − | + | + | + |
| ARDS | + | − | − | − | − | + |
| Biochemistry | ||||||
| Haemoglobin (g/l) | 119 | 87 | 93 | 110 | 94 | 59 |
| Leucocyte (counts/mm3) | 6800 | 27,600 | 2700 | 4500 | 13730 | 1,470 |
| PMN count/mm3 | 3800 | 25000 | 1100 | 3250 | 10280 | 830 |
| Lymphocyte count/mm3 | 923 | 1400 | 860 | 540 | 1250 | 500 |
| Platelet count/mm3 | 25000 | 352000 | 98000 | 60000 | 560000 | 17000 |
| Hyperbasophilic form | 30% | — | 23% | — | — | — |
| ALT/AST, U/l (<20/41)* | 3420/1630 | 37/60 | 33/56 | 38/61 | 52/18 | 51/127 |
| LDH, U/l (<280)* | 3600 | 1000 | 550 | 740 | 350 | 3026 |
| Ferritin, µg/l (15–300)* | 210000 | 11470 | 71600 | 17440 | 645 | 26000 |
| Glycosylated ferritin (>50%)* | 35% | <5% | ND | <5% | 10% | 6% |
| Triglycerides, mmol/l (<1.6)* | 3.27 | 1.9 | 3.64 | 1.76 | 3.85 | 4.24 |
| ESR, mm/h | 14 | 80 | ND | 50 | 116 | 70 |
| CRP, mg/l (<5)* | 214 | 355 | 39 | 393 | 259 | 154 |
| DIC | + | − | − | − | − | + |
| ANA | − | 1:320 | − | − | − | 1:80 |
| Possible target agent | − | − | Gold salts | − | − | ATB |
| Treatment | High‐dose CS, IVIG | High‐dose CS, MTX, IVIG | G‐CSF stop gold salts | CS | IVIG | Resuscitation for overwhelming shock and DIC high‐dose CS stop ATB |
| Outcome | Death | Remission (within 1.5 mo) | Remission (10 d) | Remission (4 d) | Remission (20 d) | Remission (10 d) |
ANA, antinuclear antibodies; AOSD, adult‐onset Still's disease; ARDS, acute respiratory distress syndrome; ATB, anti‐tuberculosis treatment; CRP, C‐reactive protein; CS, corticosteroids; d, days; DIC, disseminated intravascular coagulopathy; ESR, erythrocyte sedimentation rate; G‐CSF, granulocyte cell stem factor; IS, immunosuppressive; IVIG, intravenous immunoglobulin; LDH, lactate dehydrogenase; mo, months; MTX, methotrexate; ND, not determined; PMN, polymorphonuclear neutrophil; RHS, reactive haemophagocytic syndrome; y, years; −, finding was absent; +, finding was present; .
*Reference ranges for normal values of biological parameters are indicated in parentheses.