Table 1

Pre- and post-transplant course of disease and immunosuppressive treatment

Age at HSCT (years)	Sex/nationality	Major symptoms at point of primary diagnosis	Developing complications	Pretransplant immunosuppressive treatments		Reason for HSCT	Duration of disease until HSCT (months)	Stem cell mobilisation with:	Leukapheresis	Enrichment ex vivo with CD34+ cells	Conditioning regimen	HSCT	Course after HSCT	Response and outcome	Current immuno-suppressive drugs
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	Haemoptysis, pulmonary artery aneurysms	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	Yes	Melphalan (200 mg/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)