Table 1.
Immunological and clinical parameters
| Parameters | Pretransplant | Post-transplant | Reference range |
| Laboratory | |||
| Haemoglobin (g/dL) | 9.8 | 12.4 | 13.5–17.5 |
| Leucocytes (109/L) | 1.88 | 3.47 | 150–450 |
| Lymphocytes (109/L) | 0.17 | 1.31 | 1.2–5.2 |
| Neutrophils (109/L) | 1.52* | 1.79 | 1.8–8.0 |
| Monocytes (109/L) | 0.19 | 0.37 | 0.2–0.8 |
| Platelets (109/L) | 29 | 183 | 150–400 |
| CD3+ (cells/µL) | 800 | 1914 | 800–3500 |
| CD8+ (cells/µL) | 554 | 936 | 200–1200 |
| CD4+ (cells/µL) | 238 | 920 | 400–1200 |
| CD56+ (cells/µL) | 35 | 99 | 70–1200 |
| CD19+ (cells/µL) | 138 | 99 | 200–600 |
| Activated T cells (HLA-DR+ %) |
55 | 25 | N/A |
| CD4+ naive (%) | Not detected | 244 | N/A |
| CD27– IgD+ (naive) (%) | 87 | 93 | 75.2–86.7 |
| CD27+ IgD+ (memory) (%) | 9 | 4 | 4.6–10.2 |
| CD27+ IgD– (class-switched) (%) |
2 | 3 | 3.3–9.6 |
| IgM (g/L) | 0.55 | 0.25 | 0.50–1.90 |
| IgG (g/L) | 6.4 | 8.2 | 5.4–16.1 |
| IgA (g/L) | 0.92 | 0.33 | 0.80–2.80 |
| Tetanus (IU/mL) | 0.93 | ND | 0.1–10 |
| Haemophilus influenzae b (mg/mL) | 1.8 | ND | 1.0–20.0 |
| Pneumococcal (mg/mL) | 10 | ND | 20–200 |
| Anti-GAD antibody (IU/mL) | >2000 | >2000 | 0–9.9 |
| Islet cell antibody | Detected | Detected | N/A |
| pANCA | Detected | Detected | N/A |
| Clinical | |||
| FEV1 (% predicted) | 38 | 84 | 95–100 |
*Peripheral neutrophils were supported pretransplant by recombinant granulocyte colony stimulating factor. Post-transplant parameters were obtained at 18 months (FBC and T-cell indices, lung function) or 21 months post-HSCT (B cell and antibody indices). Post-HSCT antibody indices were measured during concomitant subcutaneous immunoglobulin supplementation. No other autoantibodies were detected pre-HSCT or post-HSCT.
FBC, full blood count; FEV1, forced expiratory volume in 1 s; GAD, glutamic acid decarboxylase; HLA-DR, human leucocyte antigen–antigen D related; HSCT, haematopoietic stem cell transplantation; ND, not done; pANCA, perinuclear anti neutrophil cytoplasmic antibody.