Clinical Features of Study Population (45 XHIM males from 30 unrelated families)
| Age at Onset* | Age When Ig Therapy Started | ||
| Age (yr) | No. of Patients | Age (yr) | No. of Patients |
| <1 | 24 (59%) | <1 | 24 (53%) |
| 1-3 | 10 (24%) | 1-3 | 11 (24%) |
| 4-5 | 4 (10%) | 4-5 | 2 |
| 8 | 1 | 6-10 | 2 |
| 14 | 1 | 11-15 | 3 |
| 17 | 1 | 16-20 | 1 |
| Never | 2† | ||
| PCP 17 Patients (38%) | Neutropenia 27 Patients (60%) | ||
| Age (yr) | No. of Patients | No. of Patients | |
| <1 | 14 | Transient | 12 |
| 1-4 | 3‡ | Intermittent | 3 |
| Chronic | 11 | ||
| Associated with B19 infection | 12-153 | ||
| Cholangiolitis and cirrhosis (due to Cryptosporidiuminfection)4 patients (9%) | |||
| Malignancy | 5 Patients (11%) | ||
| Response to IVIg Therapy2-155 | |||
| No. of patients | |||
| Good | 20 (48%) | ||
| Fair | 17 (41%) | ||
| Marginal | 5 (12%) | ||
| Age at Onset* | Age When Ig Therapy Started | ||
| Age (yr) | No. of Patients | Age (yr) | No. of Patients |
| <1 | 24 (59%) | <1 | 24 (53%) |
| 1-3 | 10 (24%) | 1-3 | 11 (24%) |
| 4-5 | 4 (10%) | 4-5 | 2 |
| 8 | 1 | 6-10 | 2 |
| 14 | 1 | 11-15 | 3 |
| 17 | 1 | 16-20 | 1 |
| Never | 2† | ||
| PCP 17 Patients (38%) | Neutropenia 27 Patients (60%) | ||
| Age (yr) | No. of Patients | No. of Patients | |
| <1 | 14 | Transient | 12 |
| 1-4 | 3‡ | Intermittent | 3 |
| Chronic | 11 | ||
| Associated with B19 infection | 12-153 | ||
| Cholangiolitis and cirrhosis (due to Cryptosporidiuminfection)4 patients (9%) | |||
| Malignancy | 5 Patients (11%) | ||
| Response to IVIg Therapy2-155 | |||
| No. of patients | |||
| Good | 20 (48%) | ||
| Fair | 17 (41%) | ||
| Marginal | 5 (12%) | ||
*Four unrelated patients (SC [family 3], AY [family 5], JaB [family 9], and RoC [family 22], each with an older affected brother) are excluded since they were diagnosed before developing symptoms.
JoC (family 22) died of PCP at 8 months of age. IN (family 8), now 24 years of age, is doing well without IVIg infusion.
SC (family 3), AY (family 5), and JaB (family 9) developed PCP after trimethoprim-sulfamethoxazole prophylaxis had been stopped.
Neutropenia developed during parvovirus B19 infection (DJ, family 11).
Three patients cannot be evaluated and were excluded; MS (family 20) underwent bone marrow transplantation at an early age; IN (family 8) was never treated with IVIg; and JoC (family 22) died of PCP at 8 months of age. Clinical course after initiation of IVIg therapy was evaluated according to the following criteria: good, frequency and severity of infection similar to those expected in normal children; fair, infections are not severe but more often than in normal children and less frequent than before IVIg; and marginal, frequency and severity of common infections seem improved, but the clinical symptoms may at times be severe.