Page 120 - 2020_02-Haematologica-web

G. Goyal et al.
went subsequent surgery, and three (13%) received sys- temic therapy. The most common site of RDD in patients who underwent surgery was subcutaneous nodules (13 or 54%), with other single cases of isolated thyroid, bone, breast, lacrimal gland, nasal septum and dura involve-
ment, respectively. Among the five patients who required subsequent surgery, one had a nasal septal mass that recurred, while three had other disease sites (bone, soft tissue, subcutaneous tissue) that required resection subse- quently. Additionally, there was one patient with KRAS
Table 2. Treatments and overall response rates (ORR) in patients with Rosai-Dorfman disease.
Treatment First line
Surgery 24
Surgery + RT 1 Corticosteroids 17 Rituximab 2 Observation 8
ORR
24 (100%)
1 (100%) 10 (56%) 2 (100%) 0
0
2 (100%)
−
−
−
0
−
−
−
−
−
2nd/later line
7
1 3 1
4 1 1 6 1 1 3 1 1 1 1
ORR
6 (100%)
1 (100%) 2 (67%) 1 (100%)
1(25%) 1 (100%) 1 (100%) 4 (67%) 0
0
2 (100%) 1 (100%) 0
1 (100%) 0
RT
Prednisone + 6-MP/azathioprine
CVP
Cladribine
Mycophenolate
Etoposide + Vinblastine + prednisone
Prednisone + MTX/6-MP
Vinblastine + prednisone + 6-MP + MTX
Clofarabine + vinblastine + etoposide + prednisone
Pegylated-interferon
Hydroxyurea
2
2
−
−
−
1
−
−
−
−
−
RT:Radiationtherapy;6-MP:6-Mercaptopurine;CVP: cyclophosphamide,vincristine,prednisone;MTX:methotrexate
Figure 6. Treatments and outcomes of patients with Rosai-Dorfman disease (RDD) from diagnosis until first response where available. 6MP: 6-mercaptopurine; CVP: cyclophosphamide, vincristine, prednisone; 2-CDA: cladribine; MTX: methotrexate
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haematologica | 2020; 105(2)