Table 2.
Clinical data and previous findings pertaining to the SchS patients included in the IgH repertoire sequencing work.
| ID | Sex | Age at symptom onset (years) | Duration of ShcS related symptoms (years) | CRP levels (mg/dl) | Para-protein | IgMk para-protein levels (g/l) | Response to IL-1 inhibition | Bone marrow histology |
Genes/Variants identified by MDS panel | MYD88 L265P? |
|---|---|---|---|---|---|---|---|---|---|---|
| 4 | male | 36.8 | 16.8 | 4.0 | IgMk | 3 | Partial | No overt LPL | No | |
| 5 | male | 43.9 | 8.7 | 6.0 | IgGλ | N/A | complete | 15% plasma cell | No | |
| 6 | female | 44.8 | 17.1 | 12.0 | IgMk | 1 | complete | No overt LPL | No | |
| 7 | female | 49.6 | 8.4 | 8.9 | IgMk | 4 | complete | not done | No | |
| 8 | male | 52.8 | 15.3 | 4.5 | IgMλ (IF) | N/A | complete | No overt LPL | No | |
| 9 | male | 58.1 | 14.4 | 4.9 | IgMk | 3 | complete | LPL |
STAG-2 c.559C>T p.Gln187* Predicted: pathogenic VAF: 0.081 |
No |
| 10 | male | 59.6 | 13.3 | 7.9 | IgMλ | 7 | died before treatment | No overt LPL | No | |
| 11 | male | 61.7 | 13.3 | Not avail | IgMk | 5 | complete | No overt LPL | No | |
| 12 | female | 68.4 | 21.3 | 14.0 | IgMk | 8 | complete | No overt LPL | No | |
| 13 | male | 78.9 | 13.6 | 14.3 | IgMk | 7 | complete | No overt LPL | L265P |
IF, Immunofluorescence; VAF, Variant allele frequency; LPL, lymphoproliferative lymphoma.
*here is used as a standard way to mark the effects of mutation.