Table 1.
IVCY Group | |||||||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Microorganisms | Clinical Manifestations and Organ Involvements | ||||||||||||||||
Case No. | Gender | Age at Disease Onset, y | NTM | Other | Prolonged Fever | Weight Loss | Lymphadenopathy | Skin | Pulmonary | Septicemia | Musculoskeletal | Miscellaneous | Duration From Disease Onset to IGA Diagnosis, d | Duration From Disease Onset to Enrollment, d | Prior Treatment | Cumulative Dose of IVCY/ Cycles, mg | Outcome of Treatment |
1 | F | 35 | M. avium complex, M. fortuitum | None | X | X | X | X | X | X | X | 39 | 1926 | ABx + IVCY (NIH regimen) × 7 cycles | 11 250/13 | R | |
2 | M | 59 | M. kansasii | Salmonella spp., HSV, Cryptococcus spp. | X | X | X | X | X | X | X | X | 882 | 1747 | ABx + plasmaphe- resis × 3 d | 7500/10 | R |
3 | F | 56 | NTM | None | X | X | X | X | 81 | 540 | ABx | 4100/8 | R | ||||
4 | M | 59 | M. abscessus | HZV | X | X | X | X | X | X | 21 | 1086 | ABx | 4600/6 | R | ||
5 | M | 40 | M. abscessus | None | X | X | X | X | 571 | 1600 | ABx | 7400/10 | R | ||||
6 | F | 47 | M. manteneii | Cryptococcus spp. | X | X | X | X | X | X | X | 158 | 907 | ABx | 3350/6 | PR | |
7 | F | 45 | M. parascofula- ceum, M. abscessus | None | X | X | X | X | X | X | X | 53 | 835 | ABx + IVCY (NIH regimen) × 6 cycles | 3650/5 | PR | |
8 | M | 62 | M. abscessus | Salmonella spp., HSV, Cryptococcus spp. | X | X | X | X | X | X | 29 | 163 | ABx | 2800/5 | R | ||
9 | M | 46 | M. abscessus | None | X | X | X | X | X | X | 59 | 659 | ABx | 6050/7 | R | ||
10 | M | 52 | M. abscessus | Cryptococcus spp., cytomegalovirus | X | X | X | X | X | X | X | 252 | 488 | ABx | 4700/6 | R | |
11 | M | 64 | M. fortuitum | Salmonella spp. | X | X | X | X | X | X | 36 | 213 | ABx | 3900/6 | R | ||
RTX Group | |||||||||||||||||
Case No. | Gender | Age at Disease Onset, y | Microorganisms | Clinical Manifestations and Organ Involvements | Duration From Disease Onset to IGA Diagnosis, d | Duration From Disease Onset to Enrollment, d | Prior Treatment | Cumulative Dose of RTX/Cycles, mg/m2 | Outcome of Treatment | ||||||||
NTM | Other | Prolonged Fever | Weight Loss | Lymphadenopathy | Skin | Pulmonary | Septicemia | Musculoskeletal | Miscellaneous | ||||||||
1 | F | 66 | None | Salmonella spp., HSV, HZV | X | X | X | X | 108 | 250 | ABx | 345/6 | PR | ||||
2 | F | 48 | M. avium complex | None | X | X | X | X | 1275 | 1997 | ABx | 339/4 | R | ||||
3 | F | 44 | M. szulgai, M. angelicum | MTB | X | X | X | X | X | 128 | 231 | ABx | 359//4 | PR | |||
4 | F | 79 | M. avium complex | None | X | 180 | 383 | ABx | 326/2 | R | |||||||
5 | M | 51 | M. avium complex | None | 279 | 574 | ABx | 319/2 | R | ||||||||
6 | M | 62 | None | Salmonella spp. | X | X | X | X | X | 174 | 876 | ABx | 286/2 | R |
Abbreviations: ABx, antibiotics; F, female; HSV, herpes simplex virus; HZV, herpes zoster virus; IGA, adult-onset immunodeficiency associated with interferon-γ autoantibody; IVCY, intravenous cyclophosphamide; M, male; MKD, mg/kg/dose; MTB, M. tuberculosis; NIH, National Institutes of Health; NTM, nontuberculous mycobacteria; PR, partial remission; R, remission; RTX, rituximab.