Characteristics of the patient population and clinical features
| . | All patients (N = 90) . | Index cases (n = 63) . | Symptomatic relatives (n = 27) . | Asymptomatic relatives (n = 47) . |
|---|---|---|---|---|
| Median age, y (range) | 22.5 (1.9-77) | 17.6 (1.9-48) | 37.5 (1.2-77) | 43.7 (5-73) |
| Sex ratio, male/female | 62/28 (2.2) | 44/19 (2.1) | 18/9 (2) | 20/27 (0.7) |
| Lymphoproliferation | ||||
| Benign chronic LP* | 89/90 (99) | 62/63 (98) | 27/27 (100) | 0 |
| Splenomegaly, % | 85/90 (95) | 59/63 (93) | 26/27 (95) | |
| Lymphadenopathy, % | 76/84 (90) | 58/63 (92) | 18/21 (85) | |
| Hepatomegaly, % | ND | 33/63 (50) | ND | |
| Autoimmunity | ||||
| Any AI symptoms, % | 55/88 (63) | 41/63 (65) | 14/25 (56) | 0 |
| AI cytopenias, % | 47/88 (53) | 34/63 (55) | 13/25 (52) | |
| Other AI symptoms, % | 13/88 (15) | 12/63 (20) | 1/25 | |
| Neoplasia | ||||
| All | 8/90 (9) | 5/63 (8) | 3/27 (11) | 0 |
| B-cell NHL | 4/90 | 3/63 | 1/27 | |
| HL | 3/90 | 1/63 | 2/27 | |
| Glioma | 1/90 | 1/63 | 0/27 | |
| . | All patients (N = 90) . | Index cases (n = 63) . | Symptomatic relatives (n = 27) . | Asymptomatic relatives (n = 47) . |
|---|---|---|---|---|
| Median age, y (range) | 22.5 (1.9-77) | 17.6 (1.9-48) | 37.5 (1.2-77) | 43.7 (5-73) |
| Sex ratio, male/female | 62/28 (2.2) | 44/19 (2.1) | 18/9 (2) | 20/27 (0.7) |
| Lymphoproliferation | ||||
| Benign chronic LP* | 89/90 (99) | 62/63 (98) | 27/27 (100) | 0 |
| Splenomegaly, % | 85/90 (95) | 59/63 (93) | 26/27 (95) | |
| Lymphadenopathy, % | 76/84 (90) | 58/63 (92) | 18/21 (85) | |
| Hepatomegaly, % | ND | 33/63 (50) | ND | |
| Autoimmunity | ||||
| Any AI symptoms, % | 55/88 (63) | 41/63 (65) | 14/25 (56) | 0 |
| AI cytopenias, % | 47/88 (53) | 34/63 (55) | 13/25 (52) | |
| Other AI symptoms, % | 13/88 (15) | 12/63 (20) | 1/25 | |
| Neoplasia | ||||
| All | 8/90 (9) | 5/63 (8) | 3/27 (11) | 0 |
| B-cell NHL | 4/90 | 3/63 | 1/27 | |
| HL | 3/90 | 1/63 | 2/27 | |
| Glioma | 1/90 | 1/63 | 0/27 | |
More than 6 months.
ND indicates not determined.