Table 4.
Characteristics of Patients Transplanted in Neonatal Period that Later Developed an Autoimmune Cytopenia
| Pt No. | Pretranplant Diagnosis | Initial GVHD Prophylaxis | Type of Cytopenia | Posttransplant Onset (Days) | RBC Antibody | Plt Antibody | Tacrolimus Exposure | Treatment | Outcome |
|---|---|---|---|---|---|---|---|---|---|
| Cytopenias without other GVHD manifestations | |||||||||
| 2 | Beta-thalassemia | CsA, Methypred | AIHA, thrombocytopenia | 131 | IgG+ panreactive | No | Methylpred, IVIG, Azathioprine | Alive. Resolved. | |
| 6 | Krabbe | CsA, Methypred | Thrombocytopenia | 279 | No | Methylpred, IVIG, d/c CSA | Alive. Resolved. | ||
| 12 | Krabbe | CsA, Methypred | AIHA | 503 | IgG+, panreactive, C3+ | No | Methylpred, IVIG, Azathioprine, Rituximab | Alive. On treatment with intermittent hemolysis. | |
| 13 | Krabbe | CsA, Methypred | AIHA | 183 | IgG+ panreactive, C3+ | Yes | Methylpred, Azathioprine, Rituximab, d/c CSA | Alive. Resolved. | |
| 15 | Krabbe | CsA, Methypred | AIHA, thrombocytopenia, neutropenia | 132 | IgG+, panreactive, C3+ | Yes | Yes | Methylpred, Azathioprine, Rituximab, EPO, GCSF, d/c CSA | Alive. Continues on azathioprine (weaning). |
| 19 | Krabbe | CsA, MMF | AIHA | 320 | IgG+, panreactive (Anti-e, C) | No | Methylpred, Azathioprine, Rituximab | Alive. On treatment. | |
| Cytopenias with cGVHD | |||||||||
| 9 | MPS II | CsA, Methypred | AIHA, thrombocytopenia | 92 | IgG+ panreactive, C3+, (Anti-E) | Yes | Yes | Methylpred, IVIG, Azathioprine, Rituximab, Splenectomy, d/c CSA | Died of multisystem organ failure. Had secondary graft failure and underwent second transplant. |
| 10 | MPS II | CsA, Methypred | AIHA, thrombocytopenia | 283 | IgG+, C3+, (Anti-A) | Yes | Yes | Methylpred, Rituximab (daclizimab for skin GVHD) | Alive. Resolved. |
| 16 | MLD | CsA, MMF | AIHA, thrombocytopenia, neutropenia | 687 | Yes | No | Methylpred, Rituximab | Alive. Ongoing pancytopenia. Secondary graft failure requiring second transplant. | |
| 17 | Krabbe | CsA, MMF | AIHA | 215 | IgG+ panreactive, (Anti-E) | Yes | Methylpred, Azathioprine, Rituximab, d/c CSA | Alive. On azathioprine and steroids for skin cGVHD. | |
Krabbe indicates globoid cell leukodystrophy (Krabbe disease); MPS II, mucopolysaccaridosis type II (Hunter’s Syndrome); MLD, metachromatic leukodystrophy; DAT, direct antibody testing; Methylpred, methyl-prednisolone; CSA, cyclosporine; MMF, mycophenolate; EPO, erythropoietin; G-CSF, granulocyte-colony stimulating factor; GVHD, graft-versus-host disease; Pt, patient.