Clinical and immunologic profiles of 6 patients with ICL compared with healthy controls
| Characteristic . | ICL patient no. . | Healthy controls . | |||||
|---|---|---|---|---|---|---|---|
| P1 . | P2 . | P3 . | P4 . | P5 . | P6 . | ||
| Age, y | |||||||
| At time of study | 43 | 51 | 51 | 31 | 35 | 75 | 18-60 |
| At time of first infection | 42 | 50 | 48 | 30 | 33 | 72 | |
| Opportunistic infection | |||||||
| Cryptococcus neoformans | Disseminated | Meningeal | Bone | ||||
| Alternaria sp | Skin (recurrent) | ||||||
| Nocardia brasiliensis | Skin + lung | ||||||
| Mycobacterium tuberculosis | Lung | ||||||
| M kansasii | Disseminated | ||||||
| Pneumocystis jirovecii | Lung | ||||||
| IgG, g/L | 7.9 | 12.0 | 7.5 | 12.0 | 11.1 | 6.2 | 7-14 |
| IgM, g/L | 0.7 | 0.6 | 1.6 | 1.7 | 1.1 | 0.4 | 0.4-2.4 |
| IgA, g/L | 1.3 | 1.9 | 2.0 | 1.0 | 1.5 | 0.9 | 0.7-3.7 |
| Pre/post IL-2 values | |||||||
| Total Ly/mm3 | 653/857 | 390/1340 | 165/200 | 799/ND | 450/ND | 979/990 | 1742 ± 429 |
| CD4+ T Ly/mm3 (% Ly) | 20(3)/215(15) | 120(31)/582(43) | 6(4)/10(5) | 208(26)/ND | 185(41)/ND | 137(14)/247(25) | 44 ± 11 |
| CD8+ T Ly/mm3 (% Ly) | 418(64)/377(44) | 148(38)/432(32) | 2(1)/5(2) | 228(28)/ND | 81(18)/ND | 206(21)/178(18) | 28 ± 7 |
| B Ly/mm3 (% Ly) | < 1/< 1 | 27(7)/ND | < 1/< 1 | 40(5)/ND | 9(2)/ND | 176(18)/198(20) | 17 ± 9 |
| γδ T Ly/mm3 (% Ly) | 144(22)/214(25) | 47(12)/ND | 46(28)/ND | 119(15)/ND | 71(15)/ND | 117(12)/40(4) | 3.5 ± 2 |
| NK Ly/mm3 (% Ly) | 79(12)/215(15) | 63(16)/ND | 102(60)/ND | 144(18)/ND | 104(22)/ND | 343(35)/327(33) | 9.5 ± 4.8 |
| CD4+ T Ly surface levels, % | |||||||
| CXCR4 | < 3 | 12 | < 3 | 8 | < 3 | 4.3 | 25 ± 5 |
| CCR5 | 15 | 8 | 22 | 17 | 12 | 17.5 | 16 ± 8 |
| CD8+ T Ly surface levels, % | |||||||
| CXCR4 | 4 | 9.7 | 3 | 10.4 | 1 | 10.5 | 19 ± 8 |
| CCR5 | 11 | 8 | 18 | 21 | 8 | 22 | 12 ± 15 |
| CD4+ T Ly intracytoplasmic levels, % | |||||||
| CXCR4 | 56 | 35 | 47 | 32 | 52 | 29 | 2.0 ± 0.6 |
| CCR5 | 3 | 7 | 5 | 2 | 1 | 3 | 0 |
| CXCL12 | 69 | 38 | 42 | 22 | 34 | 32 | 0 |
| CD8+ T Ly intracytoplasmic levels, % | |||||||
| CXCR4 | 9 | 12 | < 1 | 2 | 8 | 3 | < 1.0 |
| CCR5 | 1 | 2 | < 1 | 0 | 0 | 0 | 0 |
| CXCL12 | 1 | 2 | 2 | 1 | 1 | 1 | 0 |
| Characteristic . | ICL patient no. . | Healthy controls . | |||||
|---|---|---|---|---|---|---|---|
| P1 . | P2 . | P3 . | P4 . | P5 . | P6 . | ||
| Age, y | |||||||
| At time of study | 43 | 51 | 51 | 31 | 35 | 75 | 18-60 |
| At time of first infection | 42 | 50 | 48 | 30 | 33 | 72 | |
| Opportunistic infection | |||||||
| Cryptococcus neoformans | Disseminated | Meningeal | Bone | ||||
| Alternaria sp | Skin (recurrent) | ||||||
| Nocardia brasiliensis | Skin + lung | ||||||
| Mycobacterium tuberculosis | Lung | ||||||
| M kansasii | Disseminated | ||||||
| Pneumocystis jirovecii | Lung | ||||||
| IgG, g/L | 7.9 | 12.0 | 7.5 | 12.0 | 11.1 | 6.2 | 7-14 |
| IgM, g/L | 0.7 | 0.6 | 1.6 | 1.7 | 1.1 | 0.4 | 0.4-2.4 |
| IgA, g/L | 1.3 | 1.9 | 2.0 | 1.0 | 1.5 | 0.9 | 0.7-3.7 |
| Pre/post IL-2 values | |||||||
| Total Ly/mm3 | 653/857 | 390/1340 | 165/200 | 799/ND | 450/ND | 979/990 | 1742 ± 429 |
| CD4+ T Ly/mm3 (% Ly) | 20(3)/215(15) | 120(31)/582(43) | 6(4)/10(5) | 208(26)/ND | 185(41)/ND | 137(14)/247(25) | 44 ± 11 |
| CD8+ T Ly/mm3 (% Ly) | 418(64)/377(44) | 148(38)/432(32) | 2(1)/5(2) | 228(28)/ND | 81(18)/ND | 206(21)/178(18) | 28 ± 7 |
| B Ly/mm3 (% Ly) | < 1/< 1 | 27(7)/ND | < 1/< 1 | 40(5)/ND | 9(2)/ND | 176(18)/198(20) | 17 ± 9 |
| γδ T Ly/mm3 (% Ly) | 144(22)/214(25) | 47(12)/ND | 46(28)/ND | 119(15)/ND | 71(15)/ND | 117(12)/40(4) | 3.5 ± 2 |
| NK Ly/mm3 (% Ly) | 79(12)/215(15) | 63(16)/ND | 102(60)/ND | 144(18)/ND | 104(22)/ND | 343(35)/327(33) | 9.5 ± 4.8 |
| CD4+ T Ly surface levels, % | |||||||
| CXCR4 | < 3 | 12 | < 3 | 8 | < 3 | 4.3 | 25 ± 5 |
| CCR5 | 15 | 8 | 22 | 17 | 12 | 17.5 | 16 ± 8 |
| CD8+ T Ly surface levels, % | |||||||
| CXCR4 | 4 | 9.7 | 3 | 10.4 | 1 | 10.5 | 19 ± 8 |
| CCR5 | 11 | 8 | 18 | 21 | 8 | 22 | 12 ± 15 |
| CD4+ T Ly intracytoplasmic levels, % | |||||||
| CXCR4 | 56 | 35 | 47 | 32 | 52 | 29 | 2.0 ± 0.6 |
| CCR5 | 3 | 7 | 5 | 2 | 1 | 3 | 0 |
| CXCL12 | 69 | 38 | 42 | 22 | 34 | 32 | 0 |
| CD8+ T Ly intracytoplasmic levels, % | |||||||
| CXCR4 | 9 | 12 | < 1 | 2 | 8 | 3 | < 1.0 |
| CCR5 | 1 | 2 | < 1 | 0 | 0 | 0 | 0 |
| CXCL12 | 1 | 2 | 2 | 1 | 1 | 1 | 0 |
BM biopsies showed myelodysplasia and myelofibrosis in P3 and no abnormality in the 5 other patients.
ND indicates not done; and Ly, lymphocyte.