Table 3.
Treatment strategies for SCLS
| Therapy | Putative mechanism(s) | Efficacy in SCLS? | Reference(s) |
|---|---|---|---|
| Endothelial signal transduction | |||
| theophylline + terbutaline | phosphodiesterase inhibition,β-receptor stimulation; ↑ endothelial cAMP | Yes, acute and maintenance therapy | (59, 63) |
| epoprostenol | prostacyclin analogue; ↑ endothelial cAMP, vascular smooth muscle relaxation | Yes, in acute setting | (32) |
| HMG-coA reductase inhibitors (statins) | ↓ Rho prenylation | ND | |
| Dasatinib, imatinib | ↓Src or other tyrosine kinase activity | ND | |
| Bevacizumab | ↓VEGF activity | No (chronic form) | (14) |
| Immune modulation | |||
| Corticosteroids | anti-inflammatory | ± (chronic form of SCLS) | (10, 60–61) |
| Infliximab | ↓TNF activity | Yes, in acute setting | (14) |
| IMiDs (lenalidomide, thalidomide) | ↓plasma cell clone, anti-inflammatory mechanisms | Yes (thalidomide) | (67) |
| Plasmapheresis | ↓circulating monoclonal paraprotein | ? temporary | (53) |
| IVIG | Anti-inflammatory; anti-idiotypic mechanisms | Yes, maintenance therapy | (7, 9) |
| Hematological intervention | |||
| Autologous peripheral blood cell transplantation (PBST) | ↓plasma cell clone | ND | |
| Rituximab | ↓ B cells | ND | |
| Melphalan/prednisone | ↓plasma cell clone | Yes, in setting of myeloma, plasma cell leukemia | (49, 51) |
| Bortezomib | ↓plasma cell clone | ND | |
| Anakinra | ↓plasma cell clone | ND |
ND=no data; mAb=monoclonal antibody; IVIG=Intravenous immunoglobulin; cAMP=cyclic adenosine monophosphate