Clonal Expansion and Jβ Bias of β-T Cells in 5 FHL Patients
| Patient No. . | High Frequency (>15%) . | Clonal Expansion Vβ-Jβ4-150 . | Vβ With Jβ1 Bias (>70%)/ Investigated Vβ4-151 . | Comparison Value Jβ1:Jβ2 (αβ-T cells investigated)‡ . | |
|---|---|---|---|---|---|
| Vβ . | Vα . | ||||
| 1 (PB)4-153 | No | Not done | 13-1.2 (88%)4-155 | 13/3, 13 | 48.2:51.8 (23.9%) |
| 2 (PB) | 5a (39%), 5b (32%) | 9 (25%), 3 (20%) | 5a-1.6 (56%) | 4, 5a, 5b, 13/4, 5a, 5b, 13 | 89.7:10.3 (79.3%) |
| 3 (PB) | No | 3 (47%) | 4-1.4 (44%) | 2, 3, 4/2, 3, 4, 8, 13 | 77.4:22.6 (41%) |
| 4 (PB) | 6 (17.5%), 13 (24%) | 9 (28%) | 6-1.1 (95%), 1-1.1 (74%), 15-1.4 (98%) | 1, 4, 6, 13, 15/1, 4, 6, 13, 15 | 94.4:5.5 (64.4%) |
| 5 (BM) | 6 (61%) | 9 (15.4%) | 6-1.1 (86%) | 3, 6/3, 6, 13 | 86.6:13.4 (76%) |
| Patient No. . | High Frequency (>15%) . | Clonal Expansion Vβ-Jβ4-150 . | Vβ With Jβ1 Bias (>70%)/ Investigated Vβ4-151 . | Comparison Value Jβ1:Jβ2 (αβ-T cells investigated)‡ . | |
|---|---|---|---|---|---|
| Vβ . | Vα . | ||||
| 1 (PB)4-153 | No | Not done | 13-1.2 (88%)4-155 | 13/3, 13 | 48.2:51.8 (23.9%) |
| 2 (PB) | 5a (39%), 5b (32%) | 9 (25%), 3 (20%) | 5a-1.6 (56%) | 4, 5a, 5b, 13/4, 5a, 5b, 13 | 89.7:10.3 (79.3%) |
| 3 (PB) | No | 3 (47%) | 4-1.4 (44%) | 2, 3, 4/2, 3, 4, 8, 13 | 77.4:22.6 (41%) |
| 4 (PB) | 6 (17.5%), 13 (24%) | 9 (28%) | 6-1.1 (95%), 1-1.1 (74%), 15-1.4 (98%) | 1, 4, 6, 13, 15/1, 4, 6, 13, 15 | 94.4:5.5 (64.4%) |
| 5 (BM) | 6 (61%) | 9 (15.4%) | 6-1.1 (86%) | 3, 6/3, 6, 13 | 86.6:13.4 (76%) |
The marked bias for Jβ1 usage (the mean rate of Jβ1:Jβ2 was 87:13) was confirmed due to the calculation by densitometory in all but 1 patient (patient no. 1).
By SSCP analysis, all Vβ/Jβ fragments were suggested to be clones. All clones (7/7) used 1 member of the Jβ1 gene family. In Table 3, the sequencing of Vβ-Jβ fragments confirmed to be clones.
Vβ in each patient was investigated at random for the Jβ usage analysis. On the right side, each Vβ family member indicates the bias to Jβ1 usage. Fifteen of 19 Vβ repertoires investigated showed a bias of more than 70% Jβ1 genes usage.
For the Vβ/Jβ fragments investigated, the comparison rate (%) of Jβ1 genes/Jβ2 genes was calculated.
In all patients except patient no. 5, mononuclear cells of peripheral blood were analyzed.
Rate of 1 specific Jβ gene used in 13 types of Jβ genes.