Post-leniolisib treatment parameters
| . | Details . | T + 8 wk . | T + 12 wk . | T + 16 wk . | T + 24 wk . | T + 36 wk . |
|---|---|---|---|---|---|---|
| Auxological parameters | Weight | 35.5 kg | 35.5 kg | 36.2 kg | 38 kg | 38.2 kg |
| Height | 149.6 cm | n.a. | n.a. | 154 cm | 154 cm | |
| QoL (SF-36), % | | | | 66% | | 82% |
| Hematological | Hb level | 14.6 g/dl | 14.4 g/dl | 15.5 g/dl | 15.8 g/dl | 16.8 g/dl |
| WBC count | 5,240/mm3 | 4,970/mm3 | 4,120/mm3 | 4,600/mm3 | 5,060/mm3 | |
| Lymphocyte count | 1,938/mm3 | 2,160/mm3 | 1,830/mm3 | 2,120/mm3 | 2,300/mm3 | |
| PLT count | 158,000/mm3 | 169,000/mm3 | 165,000/mm3 | 150,000/mm3 | 192,000/mm3 | |
| Immunophenotype | CD3 | 1,656/mm3 | 1,866/mm3 | 1,541/mm3 | 1,594/mm3 | 1,435/mm3 |
| CD4 | 1,183/mm3 | 1,265/mm3 | 1,119/mm3 | 1,111/mm3 | 992/mm3 | |
| CD8 | 429/mm3 | 551/mm3 | 401/mm3 | 466/mm3 | 424/mm3 | |
| CD19 | 190/mm3 | 251/mm3 | 213/mm3 | 269/mm3 | 307/mm3 | |
| CD16-56 | 122/mm3 | 193/mm3 | 145/mm3 | 180/mm3 | 204/mm3 | |
| Naive B cell | 180/mm3 | 241/mm3 | 200/mm3 | 261/mm3 | 298/mm3 | |
| Memory B cell | 6/mm3 | 10/mm3 | 10.7/mm3 | 13.3/mm3 | 12/mm3 | |
| Transitional B cell | 15% | 15% | 5.8% | 4.7% | 1.6% | |
| Naive CD4 cell | 733/mm3 | 746/mm3 | 615/mm3 | 855/mm3 | 798/mm3 | |
| Naive CD8 cell | 197/mm3 | 28/mm3 | 180/mm3c | 89/mm3 | 224/mm3 | |
| IgG | 682 mg/dl | 686 mg/dl | 769 mg/dl | - | 979 mg/dl | |
| IgM | 67 mg/dl | 39 mg/dl | 40 mg/dl | 36 mg/dl | 32 mg/dl | |
| IgA | 31 mg/dl | 26 mg/dl | 25 mg/dl | 24 mg/dl | 30 mg/dl | |
| Lung function | DLCO-Plethysmography | | | TLC: 2.88 L RV/TLC: 31% Reduction in air trapping, plethysmography improved | | |
| Radiological CT | Thymus | | | | | Volume reduction: 44.62% |
| Lung | | | | | Resolution of consolidation bands and tree-in-bud appearance, less prominent “mosaic attenuation areas” | |
| Lymph nodes | | | | | The left axillary lymph node shows a reduction in size and postcontrast enhancement | |
| Radiological MRI | LTC lymph nodes | | Max 1.8 cm | | Max 2.1 cm | |
| Axillary lymph nodes | | n.a. | | Max 1.3 cm | | |
| Spleen | | 13 cm | | 12.5 cm (volume reduction of 52.37% vs. pre-leniolisib); the hypointense nodular area within the splenic parenchyma is no longer identifiable | | |
| Radiological US | LTC lymph nodes | | <1 cm | | <1 cm | |
| Submandibular lymph nodes | | R 18 × 6 mm | | R 12 × 4 mm L 13 × 5 mm | | |
| Axillary lymph nodes | | <1 cm | | R 17 × 3 mm L 18 × 3 mm | | |
| Subclavicular lymph nodes | | <1 cm | | <1 cm | | |
| Spleen | | 12.3 cm | | 11 cm | |
| . | Details . | T + 8 wk . | T + 12 wk . | T + 16 wk . | T + 24 wk . | T + 36 wk . |
|---|---|---|---|---|---|---|
| Auxological parameters | Weight | 35.5 kg | 35.5 kg | 36.2 kg | 38 kg | 38.2 kg |
| Height | 149.6 cm | n.a. | n.a. | 154 cm | 154 cm | |
| QoL (SF-36), % | | | | 66% | | 82% |
| Hematological | Hb level | 14.6 g/dl | 14.4 g/dl | 15.5 g/dl | 15.8 g/dl | 16.8 g/dl |
| WBC count | 5,240/mm3 | 4,970/mm3 | 4,120/mm3 | 4,600/mm3 | 5,060/mm3 | |
| Lymphocyte count | 1,938/mm3 | 2,160/mm3 | 1,830/mm3 | 2,120/mm3 | 2,300/mm3 | |
| PLT count | 158,000/mm3 | 169,000/mm3 | 165,000/mm3 | 150,000/mm3 | 192,000/mm3 | |
| Immunophenotype | CD3 | 1,656/mm3 | 1,866/mm3 | 1,541/mm3 | 1,594/mm3 | 1,435/mm3 |
| CD4 | 1,183/mm3 | 1,265/mm3 | 1,119/mm3 | 1,111/mm3 | 992/mm3 | |
| CD8 | 429/mm3 | 551/mm3 | 401/mm3 | 466/mm3 | 424/mm3 | |
| CD19 | 190/mm3 | 251/mm3 | 213/mm3 | 269/mm3 | 307/mm3 | |
| CD16-56 | 122/mm3 | 193/mm3 | 145/mm3 | 180/mm3 | 204/mm3 | |
| Naive B cell | 180/mm3 | 241/mm3 | 200/mm3 | 261/mm3 | 298/mm3 | |
| Memory B cell | 6/mm3 | 10/mm3 | 10.7/mm3 | 13.3/mm3 | 12/mm3 | |
| Transitional B cell | 15% | 15% | 5.8% | 4.7% | 1.6% | |
| Naive CD4 cell | 733/mm3 | 746/mm3 | 615/mm3 | 855/mm3 | 798/mm3 | |
| Naive CD8 cell | 197/mm3 | 28/mm3 | 180/mm3c | 89/mm3 | 224/mm3 | |
| IgG | 682 mg/dl | 686 mg/dl | 769 mg/dl | - | 979 mg/dl | |
| IgM | 67 mg/dl | 39 mg/dl | 40 mg/dl | 36 mg/dl | 32 mg/dl | |
| IgA | 31 mg/dl | 26 mg/dl | 25 mg/dl | 24 mg/dl | 30 mg/dl | |
| Lung function | DLCO-Plethysmography | | | TLC: 2.88 L RV/TLC: 31% Reduction in air trapping, plethysmography improved | | |
| Radiological CT | Thymus | | | | | Volume reduction: 44.62% |
| Lung | | | | | Resolution of consolidation bands and tree-in-bud appearance, less prominent “mosaic attenuation areas” | |
| Lymph nodes | | | | | The left axillary lymph node shows a reduction in size and postcontrast enhancement | |
| Radiological MRI | LTC lymph nodes | | Max 1.8 cm | | Max 2.1 cm | |
| Axillary lymph nodes | | n.a. | | Max 1.3 cm | | |
| Spleen | | 13 cm | | 12.5 cm (volume reduction of 52.37% vs. pre-leniolisib); the hypointense nodular area within the splenic parenchyma is no longer identifiable | | |
| Radiological US | LTC lymph nodes | | <1 cm | | <1 cm | |
| Submandibular lymph nodes | | R 18 × 6 mm | | R 12 × 4 mm L 13 × 5 mm | | |
| Axillary lymph nodes | | <1 cm | | R 17 × 3 mm L 18 × 3 mm | | |
| Subclavicular lymph nodes | | <1 cm | | <1 cm | | |
| Spleen | | 12.3 cm | | 11 cm | |
DLCO, diffusing capacity of the lungs for carbon monoxide; Hb, hemoglobin; L, left; LTC, laterocervical; NA, not applicable; PLT, platelet; QoL, quality of life; R, right; RV, residual volume; SF-36, 36-Item Short Form Health Survey; T, time; TLC, total lung capacity; US, ultrasound; WBC, white blood cell.