Table 1.
Clinical, biological and monoclonal λ LC characteristics of POEMS syndrome patients
| Patient ID | BM clonal plasmocytosis | % BM plasma cells (abnormal: yes/no) | BM typing flow cytometry | BM biopsy | Bone lesions | SANGER analysis clone, yes/no | NGS analysis clone, yes/no | IGLV gene | IGLJ gene | Mutation | Monoclonal Ig (electrophoresis +immunofixation) | λFLC, mg/L (range, 5.70-26.3) | kFLC/λFLC ratio (range, 0.26-1.65) | VEGF in pg/mL (Nl <500 pg/L) | IGVH gene or other IGLV clone |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| BM sequencing | |||||||||||||||
| p4 | Negative | 2 (no) | No clone | ND | Yes | Yes | Yes | IGLV1-44 | IGLJ3*02 | 38→P | αλ | 15.8 | 1 | 1618 | IGHV3-11 and IGHV4-39 |
| p6 | Negative | 0.7 (no) | No clone | No clone | Yes | Yes | ND | IGLV1-44 | IGLJ3*02 | 38→P | ɣλ | 10.8 | 1.72 | 420 | |
| p9 | Negative | 1.5 (no) | No clone | ND | Yes | No | Yes | IGLV1-44 | IGLJ3*02 | 38→P | ɣλ | 23.3 | 0.69 | 162 | Other clone (IGLV3-1/IGLJ2*01) |
| p10 | Negative | 2 (no) | No clone | ND | Yes | Yes | Yes | IGLV1-44 | IGLJ3*02 | 38→P | αλ | 5.78 | 1.08 | 430 | IGHV3-9 |
| p11 | Negative | 2.5 (no) | No clone | ND | Yes | No | Yes | IGLV1-44 | IGLJ3*02 | 38→P | ɣλ | 100 | 0.75 | 3752 | |
| p13 | Negative | 2.5 (no) | No clone | ND | Yes | No | Yes | IGLV1-44 | IGLJ3*02 | 38→P | αλ | 161 | 0.59 | 63 332 | |
| p14 | Negative | 1.3 (no) | ND | No clone | Yes | No | Yes | IGLV1-44 | IGLJ3*02 | 38→P | αλ | 32 | 0.74 | 2300 | |
| p17 | Negative | <5 (no) | No clone | No clone | No | Yes | Yes | IGLV1-44 | IGLJ3*02 | 38→N | αλ | — | — | 2000 | |
| p1 | Positive (λ) | 0.5 (yes) | λ clone | λ clone | Yes | Yes | ND | IGLV1-44 | IGLJ3*02 | 38→P | αλ | 26.3 | 0.53 | 1965 | IGHV4-38-2 |
| p2 | Positive (λ) | 13 (yes) | λ clone | ND | Yes | Yes | Yes | IGLV1-44 | IGLJ3*02 | 38→P | ɣλ | 104 | 0.13 | 1082 | IGHV2-26 |
| p3 | Positive (λ) | 9 (yes) | λ clone | ND | Yes | Yes | ND | IGLV1-44 | IGLJ3*02 | 38→P | αλ | 35.4 | 0.38 | 7000 | |
| p5 | Positive (λ) | 3.5 (no) | λ clone | ND | No | No | Yes | IGLV1-44 | IGLJ3*02 | 38→P | αλ | 217 | 0.06 | 2725 | |
| p12 | Positive (PND) | 35 (yes) | ND | ND | No | No | Yes | IGLV1-44 | IGLJ3*02 | 38→S | αλ | 57.5 | 0.82 | 7800 | |
| p15 | Positive (λ) | 6 (yes) | λ clone | ND | Yes | Yes | Yes† | IGLV1-44 | IGLJ3*02 | 38→A | αλ | 72 | 0.21 | 9055 | |
| p16 | Positive (λ) | 5 (unknown) | λ clone | ND | No | Yes | Yes | IGLV1-44 | IGLJ3*02 | 38→A | ɣλ | 51.9 | 0.25 | 739 | |
| p23 | Negative | <5 (no) | No clone | No clone | Yes | No | Yes | IGLV1-40 | IGLJ3*02 | 40→N | No monoclonal Ig | 46 | 0.76 | 845 | |
| p24 | Negative | 4 (no) | ND | ND | Yes | Yes | Yes | IGLV1-40 | IGLJ3*02 | 40→N | — | 47 | 0.5 | 1300 | |
| p20 | Positive (k) | 2.3 (yes) | κ clone | ND | Yes | No | Yes | IGLV1-40 | IGLJ3*02 | 40→N | ɣλ and ɣk | 150 | 0.55 | 1400 | |
| p19 | Positive (PND) | 2 (yes) | λ clone | ND | No | Yes | Yes | IGLV1-40 | IGLJ3*02 | 40→N | αλ | 43.3 | 0.61 | 11 188 | |
| p21 | Positive (PND) | 22 (yes) | ND | ND | Yes | Yes | ND | IGLV1-40 | IGLJ3*02 or 01 or IGLJ2*01 | 40→Q | ɣλ | 134 | 0.09 | 1423 | |
| p22 | Positive (λ) | 3 (yes) | λ clone | ND | No | Yes | Yes | IGLV1-40 | IGLJ3*02 | 40→N | αλ | 32 | 0.74 | 7290 | IGHV2-70 |
| p25 | Positive (λ) | 0 (no) | λ clone | No clone | No | Yes | Yes | IGLV1-36 | IGLJ3*02 | — | ɣλ | 37.8 | 0.72 | 5000 | Other clone (IGLV2-14/IGLJ3*02) |
| p26 | Positive (λ) | 1 (no) | ND | λ | No | Yes | Yes | IGLV1-36 | IGLJ3*02 | — | No monoclonal Ig | 107 | 0.36 | 7550 | |
| p29 | Negative | 1% (no) | No clone | No clone | Yes | No | Yes | IGLV3-1 | IGLJ2*01 | — | ɣλ | 40.2 | 0.75 | 3275 | |
| p30 | Negative | <5 (no) | ND | No clone | No | No | Yes | IGLV3-25 | IGLJ1*01 | — | ɣλ | — | — | 1966 | |
| p27 | Positive (λ) | 2 (no) | λ clone | ND | Yes | No | Yes | IGLV3-1 | IGLJ3*01 | — | ɣλ | 35 | 0.45 | 1210 | |
| p31 | Negative | 1%-2.5% (no) | No clone | ND | Yes | No | No | No clone | No clone | — | No monoclonal Ig | 41 | 0.69 | 1700 | |
| p32 | Negative | 2 (no) | ND | ND | Yes | No | No | No clone | No clone | — | ɣλ | 51.3 | 0.46 | 1190 | |
| p33 | Negative | 3.5 (no) | No clone | ND | No | No | No‡ | No clone‡ | No clone‡ | — | ɣλ | 25.2 | 1.31 | 1146 | |
| p34 | Negative | 0.3 (no) | No clone | ND | Yes | No | No | No clone | No clone | — | ɣλ | 21.8 | 0.54 | 705 | |
| p35 | Negative | 2 (no) | No clone | ND | Yes | ND | No | No clone | No clone | — | αλ | — | — | 2550 | |
| p36 | Negative | 2 (no) | ND | ND | No | No | No | No clone | No clone | — | αλ | 27 | 1.33 | 665 | |
| Bone lesion sequencing | |||||||||||||||
| p7 | Negative | 2 (no) | ND | ND | Yes | Yes | Yes | IGLV1-44 | IGLJ3*02 | 38→A | ɣλ | 15.3 | 0.77 | 1419 | |
| p8 | Negative | 1.5 (no) | No clone | ND | Yes | Yes | Yes | IGLV1-44 | IGLJ3*02 | 38→A | ɣλ and αk | 55.6 | 0.99 | 3560 | |
| p18 | Positive (k) | ND | ND | κ | Yes | Yes | Yes | IGLV1-44 | IGLJ3*02 | 38→P | ɣk and ɣλ | 21.6 | — | 1100 |
ND, not done; PND, phenotyping not done.
†
Sequencing on both BM aspirates and bone lesions.
‡
Clone found on lymph nodes.