Literature data
| . | HX . | NX . | NXG . | Total . | |||
|---|---|---|---|---|---|---|---|
| Literature . | Present series . | Literature . | Present series . | Literature . | Present series . | ||
| No. of cases | 37 | 2 | 63 | 11 | 130 | 11 | 254 |
| Mean age, y (range) | 53.9 (31-80) | 64.5 (55-74) | 62.05 (37-83) | 56.9 (37-75) | 56.23 (17-88) | 65 (48-78) | 59.76 |
| Sex ratio (female/male) | 1.2 (19/17) | 1 (1/1) | 1 (31/32) | 0.2 (2/9) | 1.5 (78/52) | 2.7 (8/3) | 1.2 (139/114) |
| Palpebral lesions | 9/37 | 2/2 | 32/63 | 10/11 | 94/130 | 8/11 | 155/254 (61%) |
| Extracutaneous lesions | 8/37 | 1 | 8/63 | 1/11 | 23/130 | 6/11 | 47/254 (18.5%) |
| Cryoglobulinemia | 5/8 | 0/2 | 5/31 | 2/8 | 10/26 | 0/5 | 22/80 (27.5%) |
| MIg isotype | 14 IgG | 2 IgG | 56 IgG (89%) 1 IgMκ, 1 IgM λ 1 IgA, 1 BJ κ, 4 UK | 11 IgG | 105 IgG (81%), 4 IgA, 2 IgM, 8 UK | 11 IgG | 200 IgG (79%) |
| Associated gammopathy | 31 MM 4 MGUS 1 CLL 1 Waldenström | 2 MM | 30 MM, 26 MGUS, 2 MMCL, 1 CLL, 1 NHL, 2 amyloidosis | 2 MM, 8 MGUS, 1 Waldenström | 20 MM, 68 MGUS, 2 CLL, 3 NHL, 1 HL, 22 UK | 4 MM, 6 MGUS, 1 NHL | 90 MM (35.4%), 111 MGUS (44%) |
| Low serum C4 fraction < 25% of normal values | 1/2 | 2/2 | 26/31 | 9/10 | 23/33 | 10/10 | 71/88 (81%) |
| 0/1 | 2/2 | 15/26 | 8/9 | 6/14* | 7/10 | 38/62 (61%) | |
| Low C1 inhibitor level | 0/1 | NA | 12/21 | 2/6 | 4/6 | 2/4 | 20/38 (53%) |
| . | HX . | NX . | NXG . | Total . | |||
|---|---|---|---|---|---|---|---|
| Literature . | Present series . | Literature . | Present series . | Literature . | Present series . | ||
| No. of cases | 37 | 2 | 63 | 11 | 130 | 11 | 254 |
| Mean age, y (range) | 53.9 (31-80) | 64.5 (55-74) | 62.05 (37-83) | 56.9 (37-75) | 56.23 (17-88) | 65 (48-78) | 59.76 |
| Sex ratio (female/male) | 1.2 (19/17) | 1 (1/1) | 1 (31/32) | 0.2 (2/9) | 1.5 (78/52) | 2.7 (8/3) | 1.2 (139/114) |
| Palpebral lesions | 9/37 | 2/2 | 32/63 | 10/11 | 94/130 | 8/11 | 155/254 (61%) |
| Extracutaneous lesions | 8/37 | 1 | 8/63 | 1/11 | 23/130 | 6/11 | 47/254 (18.5%) |
| Cryoglobulinemia | 5/8 | 0/2 | 5/31 | 2/8 | 10/26 | 0/5 | 22/80 (27.5%) |
| MIg isotype | 14 IgG | 2 IgG | 56 IgG (89%) 1 IgMκ, 1 IgM λ 1 IgA, 1 BJ κ, 4 UK | 11 IgG | 105 IgG (81%), 4 IgA, 2 IgM, 8 UK | 11 IgG | 200 IgG (79%) |
| Associated gammopathy | 31 MM 4 MGUS 1 CLL 1 Waldenström | 2 MM | 30 MM, 26 MGUS, 2 MMCL, 1 CLL, 1 NHL, 2 amyloidosis | 2 MM, 8 MGUS, 1 Waldenström | 20 MM, 68 MGUS, 2 CLL, 3 NHL, 1 HL, 22 UK | 4 MM, 6 MGUS, 1 NHL | 90 MM (35.4%), 111 MGUS (44%) |
| Low serum C4 fraction < 25% of normal values | 1/2 | 2/2 | 26/31 | 9/10 | 23/33 | 10/10 | 71/88 (81%) |
| 0/1 | 2/2 | 15/26 | 8/9 | 6/14* | 7/10 | 38/62 (61%) | |
| Low C1 inhibitor level | 0/1 | NA | 12/21 | 2/6 | 4/6 | 2/4 | 20/38 (53%) |
CLL indicates chronic lymphoid leukemia; BJ, Bence Jones proteinuria; UK, unknown; MMCL, myelomonocytic chronic leukemia; NHL, non-Hodgkin lymphoma; and HL, Hodgkin lymphoma.
Corresponding to available values.