Main characteristics that distinguish the 3 forms of LCDD involving the lung
| . | Cystic lung LCDD, n = 414 * . | Systemic multivisceral LCDD n = 912-7 . | Nodular lung LCDD n = 108-13 . |
|---|---|---|---|
| Age, y (range) | 29.5 (20-37) | 55 (33-80) | 58 (33-76) |
| Sex ratio, M/F | 1/3 | 2/1 | 2/3 |
| Organ involvement | |||
| Lung | Yes (dyspnea) | Yes (asymptomatic) | Yes (asymptomatic) |
| Kidney | No | Yes (proteinuria, renal failure) | No |
| Heart | No | Yes (arrhythmias, heart failure) | No |
| Cause of death | Respiratory failure | Renal or heart failure | No death |
| Recurrence after adequate organ transplantation | No† | Yes† | Not needed |
| Monoclonal component (serum/urine) | 25% (serum free κ light chain) | 85%29 | ND |
| Primary bone marrow plasma cell proliferation | No | Yes (75%)‡ | No |
| Primary pulmonary lymphoproliferation | Yes | No | Yes (50%) |
| Characterization of monoclonal component | |||
| Clonal V heavy chain | IGHV4-34 | Not determined | Not determined |
| Clonal V light chain | κ, IGKV1 | κ, IGKV430 | κ, IGKV411 |
| Somatic mutations | No | Yes31,32 | Not determined |
| . | Cystic lung LCDD, n = 414 * . | Systemic multivisceral LCDD n = 912-7 . | Nodular lung LCDD n = 108-13 . |
|---|---|---|---|
| Age, y (range) | 29.5 (20-37) | 55 (33-80) | 58 (33-76) |
| Sex ratio, M/F | 1/3 | 2/1 | 2/3 |
| Organ involvement | |||
| Lung | Yes (dyspnea) | Yes (asymptomatic) | Yes (asymptomatic) |
| Kidney | No | Yes (proteinuria, renal failure) | No |
| Heart | No | Yes (arrhythmias, heart failure) | No |
| Cause of death | Respiratory failure | Renal or heart failure | No death |
| Recurrence after adequate organ transplantation | No† | Yes† | Not needed |
| Monoclonal component (serum/urine) | 25% (serum free κ light chain) | 85%29 | ND |
| Primary bone marrow plasma cell proliferation | No | Yes (75%)‡ | No |
| Primary pulmonary lymphoproliferation | Yes | No | Yes (50%) |
| Characterization of monoclonal component | |||
| Clonal V heavy chain | IGHV4-34 | Not determined | Not determined |
| Clonal V light chain | κ, IGKV1 | κ, IGKV430 | κ, IGKV411 |
| Somatic mutations | No | Yes31,32 | Not determined |