32 | Male/German | Fever, recurrent aphthous ulcers, polyarthritis, erythema nodosum, intracardiac thrombus | Haemoptysis, pulmonary artery aneurysms | High dose corticosteroids (up to 100 mg/day) for 48 months, methotrexate (40 mg/week IV) for 5 months, cyclophosphamide 150 mg/day and corticosteroids 250 mg/day for 5 months | | High disease activity with life threatening organ involvement (pulmonary bleeding) refractory to conventional chemotherapy | 48 | Two cycles of cyclophosphamide (2 g/m2 and 4 g/m2), G-CSF (10 μg/kg BW) | Twice: 2.9×106/kg BW, no selection possible; 7.1×106/kg BW, after selection 5.1×106/kg BW | Yes | Melphalan (200 mg/kg BW) | March 1999 | Time to achieve leucocytes >1000: 9 days; fever: 1 day; antibiotics for 2 days; 4 platelet transfusions | Complete remission | None |
49 | Male/Turkish | Fever, aphthous ulcers, posterior uveitis, Coombs’ test negative haemolytic anaemia, central nervous system involvement | Oral cyclophosphamide and corticosteroids (Fauci scheme) for 10 months | | 15 | Twice: 4.3×103/kg BW, no selection possible; 7.8×106/kg BW, after selection 3.6×106/kg BW | Nov 1999 | Time to achieve leucocytes >1000: 6 days; fever: 1 day; antibiotics for 7 days; 3 platelet transfusions | Partial response (recurrent oral and genital aphthous ulcers) | Corticosteroids (8 mg/day) |