Characteristics of invasive bacterial infections in ALPS patients
| Infection . | ALPS manifestations* . | Age of onset (y) . | Age at splenect (y) . | Age at infection (y) . | Elapsed time from splenect (y) . | Infectious agent . | Prophylaxis . | Last immunization (y) . | Ongoing treatment at time of infection . | Previous treatment . | Outcome . |
|---|---|---|---|---|---|---|---|---|---|---|---|
| Before splenectomy | |||||||||||
| Pm-1 | SM ADP ITP NHL | 0.2 | 6 | 0.8 | NA | S pneumoniae | — | None | None | None | Alive |
| P(HZ)-3 | SM ADP AIHA NHL | <0.5 | NA | 0.5 | NA | S pneumoniae | — | None | None | None | Alive |
| After splenectomy | |||||||||||
| Index cases | |||||||||||
| P-lb | SM | 3 | 13 | 27 | 14 | NI | P | 5 | None | None | Died |
| P-9 | HSM ITP | 6 | 12 | 35 | 23 | S agalactiae | None | 3 | None | None | Alive |
| P-14 | Hydrops, HSM, ADP Osteoporosis | 2.5 | 2 (×2) | S pneumoniae | P | 1.5 | 6MP | 6MP from 0.5 y, ongoing HSCT (6 y)* | Alive | ||
| Birth | 0.5 | 10.5 | 10 (×2) | NI | P | <2 | 6MP | Alive | |||
| 12.5 | 12 | S pneumoniae | P/TS/IVIG | <2 | 6MP | Alive | |||||
| P-35 | SM ADP AIHA AIN ITP | 1.5 | 3 | 14 | 11 | S pneumoniae | P | 5 | None | None | Alive |
| P-46 | SM ADP AIHA | 0.3 | 3 | 4.8 | 1.8 | S pneumoniae | P | 2 | None | CS anti-CD20** azathioprin | Died |
| Pm-16 | SM AIHA | 1.5 | 4.5 | 5.5 | 1 | S pneumoniae | None*** | 1 | None | None | Alive |
| 11 | 6.5 | S pneumoniae | Cephalo | 7.5 | None | None | Alive | ||||
| HL | 23 | 4.5 | 27 | 22.5 (×2) | S pneumoniae | None | 0.2 | None | Chemotherapy | Alive | |
| Relatives | |||||||||||
| P-4b | SM ADP | 13 | 14 | 37 | 23 | S pneumoniae | — | >5 | None | None | Alive |
| P-18b | HL | 14 | 14 | 38 | 24 | S pneumoniae | — | <5 | None | Radiotherapy chemotherapy | Died |
| P-75c | HSM ADP Anemia ITP | <2 | 2 | 4, episodes 4-6 | 2-4 | S pneumoniae | — | None | None | None | Alive |
| NHL | 38 | 46 | 44 | S pneumoniae | — | >5 | None | Axillar local radiotherapy | Alive | ||
| P-86b | SM anemia | Infancy | 6 | 34 | 28 | S pneumoniae | — | None | None | None | Died |
| Infection . | ALPS manifestations* . | Age of onset (y) . | Age at splenect (y) . | Age at infection (y) . | Elapsed time from splenect (y) . | Infectious agent . | Prophylaxis . | Last immunization (y) . | Ongoing treatment at time of infection . | Previous treatment . | Outcome . |
|---|---|---|---|---|---|---|---|---|---|---|---|
| Before splenectomy | |||||||||||
| Pm-1 | SM ADP ITP NHL | 0.2 | 6 | 0.8 | NA | S pneumoniae | — | None | None | None | Alive |
| P(HZ)-3 | SM ADP AIHA NHL | <0.5 | NA | 0.5 | NA | S pneumoniae | — | None | None | None | Alive |
| After splenectomy | |||||||||||
| Index cases | |||||||||||
| P-lb | SM | 3 | 13 | 27 | 14 | NI | P | 5 | None | None | Died |
| P-9 | HSM ITP | 6 | 12 | 35 | 23 | S agalactiae | None | 3 | None | None | Alive |
| P-14 | Hydrops, HSM, ADP Osteoporosis | 2.5 | 2 (×2) | S pneumoniae | P | 1.5 | 6MP | 6MP from 0.5 y, ongoing HSCT (6 y)* | Alive | ||
| Birth | 0.5 | 10.5 | 10 (×2) | NI | P | <2 | 6MP | Alive | |||
| 12.5 | 12 | S pneumoniae | P/TS/IVIG | <2 | 6MP | Alive | |||||
| P-35 | SM ADP AIHA AIN ITP | 1.5 | 3 | 14 | 11 | S pneumoniae | P | 5 | None | None | Alive |
| P-46 | SM ADP AIHA | 0.3 | 3 | 4.8 | 1.8 | S pneumoniae | P | 2 | None | CS anti-CD20** azathioprin | Died |
| Pm-16 | SM AIHA | 1.5 | 4.5 | 5.5 | 1 | S pneumoniae | None*** | 1 | None | None | Alive |
| 11 | 6.5 | S pneumoniae | Cephalo | 7.5 | None | None | Alive | ||||
| HL | 23 | 4.5 | 27 | 22.5 (×2) | S pneumoniae | None | 0.2 | None | Chemotherapy | Alive | |
| Relatives | |||||||||||
| P-4b | SM ADP | 13 | 14 | 37 | 23 | S pneumoniae | — | >5 | None | None | Alive |
| P-18b | HL | 14 | 14 | 38 | 24 | S pneumoniae | — | <5 | None | Radiotherapy chemotherapy | Died |
| P-75c | HSM ADP Anemia ITP | <2 | 2 | 4, episodes 4-6 | 2-4 | S pneumoniae | — | None | None | None | Alive |
| NHL | 38 | 46 | 44 | S pneumoniae | — | >5 | None | Axillar local radiotherapy | Alive | ||
| P-86b | SM anemia | Infancy | 6 | 34 | 28 | S pneumoniae | — | None | None | None | Died |
6MP, 6-mercaptopurine; ADP, adenopathy; AIHA, autoimmune hemolytic anemia; AIN, autoimmune neutropenia; anti-CD20, anti-CD20 monoclonal antibodies; cephalo, cephalosporin; CS, corticosteroids; HL, Hodgkin lymphoma; HSCT, hematopoietic sterm cell transplantation; ITP, idiopathic thrombocytopenic purpura; IVIG, intravenous immunoglobulin, NHL, non-Hodgkin lymphoma; P, penicillin; SM, spenomegaly; Splenect, splenectomy; TS, trimethoprim-sulfamethoxazol.