New classification of CVID
| CVID . | Subgroup . | No. of patients . | CD19+ B cells, % of PBLs . | CD27− IgM/D+, % of PBLs . | CD27+IgM/D+, % of PBLs . | CD27+IgM/D−, % of PBLs . | CD21−, % of B cells . | Bryant classification . | Splenomegaly . | Autoimmunity4-150 . | Vaccination4-151 . |
|---|---|---|---|---|---|---|---|---|---|---|---|
| Group I | a | 10 | 4.9 ± 2.6‡ | 3.5 ± 1.8 | 1.2 ± 0.9 | 0.1 ± 0.14-153 | 44.7 ± 11.04-153 | A/B | 10/10 (100%) | 6/10 | Neg. |
| b | 13 | 7.8 ± 3.6 | 6.5 ± 3.24-155 | 0.9 ± 0.4‡ | 0.1 ± 0.14-153 | 9.9 ± 5.7 | A/B | 5/12 (42%) | 6/13 | Interm. | |
| Group II | 7 | 12.6 ± 4.74-155 | 7.6 ± 4.3 | 3.8 ± 1.94-155 | 0.9 ± 0.44-154 | 12.6 ± 9.04-159 | C | 1/7 (14%)4-159 | 3/7 | Pos. | |
| HD | 22 | 7.7 ± 2.7 | 4.3 ± 1.6 | 1.6 ± 1.1 | 1.6 ± 0.6 | 7.0 ± 2.7 | NA | NA | NA | NA |
| CVID . | Subgroup . | No. of patients . | CD19+ B cells, % of PBLs . | CD27− IgM/D+, % of PBLs . | CD27+IgM/D+, % of PBLs . | CD27+IgM/D−, % of PBLs . | CD21−, % of B cells . | Bryant classification . | Splenomegaly . | Autoimmunity4-150 . | Vaccination4-151 . |
|---|---|---|---|---|---|---|---|---|---|---|---|
| Group I | a | 10 | 4.9 ± 2.6‡ | 3.5 ± 1.8 | 1.2 ± 0.9 | 0.1 ± 0.14-153 | 44.7 ± 11.04-153 | A/B | 10/10 (100%) | 6/10 | Neg. |
| b | 13 | 7.8 ± 3.6 | 6.5 ± 3.24-155 | 0.9 ± 0.4‡ | 0.1 ± 0.14-153 | 9.9 ± 5.7 | A/B | 5/12 (42%) | 6/13 | Interm. | |
| Group II | 7 | 12.6 ± 4.74-155 | 7.6 ± 4.3 | 3.8 ± 1.94-155 | 0.9 ± 0.44-154 | 12.6 ± 9.04-159 | C | 1/7 (14%)4-159 | 3/7 | Pos. | |
| HD | 22 | 7.7 ± 2.7 | 4.3 ± 1.6 | 1.6 ± 1.1 | 1.6 ± 0.6 | 7.0 ± 2.7 | NA | NA | NA | NA |
The new classification includes only CVID patients with peripheral B-cell numbers above 1% of PBLs. PBLs of group I CVID patients comprise less than 0.4% of class-switched memory B cells (CD27+IgM−IgD−), while in group II, like healthy donors (HD), this population accounts for more than 0.4%. Group I is further subdivided by the percentage of immature CD21− B cells into subgroups Ia (more than 20%) and Ib (less than 20%). All former group A and B patients according to the classification by Bryant et al9 fall into group I, except 2 former group B patients who fall into group II. All values are given as mean ± SE.
NA indicates not applicable.
There is a significant clustering of splenomegaly and autoimmune cytopenia in group Ia. Autoimmune phenomena in group Ia patients included 5 patients with thrombocytopenia and 1 with autoimmune hemolytic anemia; group Ib included 2 patients with pernicious anemia, 2 with vitiligo, 1 with autoimmune hemolytic anemia, and 1 with CREST syndrome; group II included 2 patients with pernicious anemia and 1 with vitiligo.
The new classification may allow a prediction of the response to vaccinations. Neg. indicates no response (n = 1); Interm., intermediate (n = 3); and Pos., normal response (n = 3) to vaccination with phage φ X174.
P < .02.
P < .0001 compared with HD.
P < .05.
P < .005 compared with HD.
One patient of group II had more than 20% immature B cells in the peripheral blood, and he was the only one with splenomegaly in this group.