Table 1.
B-cell or plasma cell neoplasms in patients with auricular chondritis or VEXAS syndrome.
| Case | Year | Number | Gender | Age of onset (years) | Diagnosis of RP | Diagnosis of VEXAS | Site of malignancy |
|---|---|---|---|---|---|---|---|
| Francès C, et al. (13) | 2001 | 2 | 1 M/1 F | Mean age: 61 | + | ? | IgA myeloma |
| Sato K, et al. (14) | 2006 | 1 | M | 51 | + | ? | Meningeal plasma cell granuloma |
| Castrejón I, et al. (15) | 2007 | 1 | M | 67 | + | ? | Lymphoplasmocytic lymphoma |
| Hayashi S, et al. (16) | 2017 | 1 | M | 77 | + | ? | Meningeal plasma cell granuloma |
| Beck DB, et al. (1) | 2020 | 4 | M | Mean age: 66 | + | + | MM or MGUS |
| Obiorah IE, et al. (5) | 2021 | 4 | M | Mean age: 63 | NA | + | MM (1/4), MGUS (1/4), MBL (1/4), MM+MBL (1/4) |
VEXAS, Vacuoles, E1 enzyme; X-linked, autoinflammatory somatic syndrome; RP, relapsing polychondritis; M, male; F, female; Ig, immunoglobulin; MM, multiple myeloma; MGUS, monoclonal gammopathy of undetermined significance; NA, not applicable; MBL, Monoclonal B-cell lymphosis.
+, is diagnosed; ?, indicates that the disease concept of VEXAS syndrome is unknown since the disease concept of VEXAS syndrome was not known at that time, and NA indicates that the diagnosis of RP is not described.