Association of clinical CVID phenotypes with BM plasma cells and nodular T-cell infiltrates
| CVID clinical phenotype* . | Splenomegaly . | Lymphadenopathy . | NLH of gut . | Granulomas . | Autoimmune cytopenia . | Other autoimmunity . |
|---|---|---|---|---|---|---|
| No. of patients† | 12 (no) vs 36 (yes) | 32 (no) vs 16 (yes) | 29 (no) vs 14 (yes) | 33 (no) vs 12 (yes) | 35 (no) vs 13 (yes) | 34 (no) vs 14 (yes) |
| BM: plasma cells‡ | NS | NS | .04‡ | NS | NS | NS |
| BM: diffuse CD3+ T-cell infiltrates§ | NS | NS | NS | NS | .04§ | NS |
| BM: nodular CD3+ T-cell infiltrates/mm biopsy§ | NS | NS | NS | NS | .014§ | NS |
| CVID clinical phenotype* . | Splenomegaly . | Lymphadenopathy . | NLH of gut . | Granulomas . | Autoimmune cytopenia . | Other autoimmunity . |
|---|---|---|---|---|---|---|
| No. of patients† | 12 (no) vs 36 (yes) | 32 (no) vs 16 (yes) | 29 (no) vs 14 (yes) | 33 (no) vs 12 (yes) | 35 (no) vs 13 (yes) | 34 (no) vs 14 (yes) |
| BM: plasma cells‡ | NS | NS | .04‡ | NS | NS | NS |
| BM: diffuse CD3+ T-cell infiltrates§ | NS | NS | NS | NS | .04§ | NS |
| BM: nodular CD3+ T-cell infiltrates/mm biopsy§ | NS | NS | NS | NS | .014§ | NS |
Data are P values of exact 2-sided Wilcoxon-Mann-Whitney test.
NS indicates not significant.
The clinical phenotype is independent of the Freiburg classification,18 which showed no significant association with BM plasma cells or with diffuse and nodular T-cell infiltrates (data not shown).
Number of patients without symptoms vs patients with symptoms.
BM plasma cells classified as absent (1), reduced (2), normal (3), increased (4). CVID patients with NLH of the gut had higher plasma cell counts than CVID patients without NLH, albeit lower than control biopsies.
Nodular CD3+ T-cell infiltrates expressed per millimeter length of BM biopsy cylinder were significantly elevated in CVID patients with autoimmune cytopenias (autoimmune hemolytic anemia, immune thrombocytopenia, or autoimmune neutropenia). The result was equally significant with the absolute number of nodules per whole biopsy, and there was a clear trend for large grade III nodules (P < .009) vs smaller ones (grade II, P < .07; and grade I, P < .06).