title: Treatment-refractory multi-lineage autoimmune cytopenia after unrelated cord blood transplantation: remission after combined bortezomib and vincristine treatment.
abstract: Autoimmune cytopenias (AC) after allogeneic hematopoietic stem cell transplantation (HSCT) are associated with a dismal prognosis. We describe a 1-year-old female with multi-lineage AC occurring on day +43 after HSCT. Multi-agent treatment with high-dose prednisolone, intravenous immunoglobulins, cyclosporine A, mycophenolate mofetil, sirolimus, and rituximab was unsuccessful. Combined treatment with bortezomib and vincristine in addition to ongoing immunosuppressive therapy was started on day +414 with transfusion-independence after day +444. Immunosuppressants were tapered until day +638. On day +1,121 the patient remained in remission. Bortezomib with vincristine may be a promising treatment modality for refractory AC after HSCT that requires further study.
authors: Division of Stem Cell Transplantation (SCT), University Children's Hospital, Zurich, Switzerland.
authors: Nicolas
authors: N
authors: Waespe
authors: Ulrike
authors: U
authors: Zeilhofer
authors: Tayfun
authors: T
authors: Güngör
mesh_terms: D001897:Boronic Acids / Q000008:administration & dosage*; D000069286:Bortezomib; D036101:Cord Blood Stem Cell Transplantation / Q000009:adverse effects*; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000628:therapy; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000188:drug therapy*; D011719:Pyrazines / Q000008:administration & dosage*; D014750:Vincristine / Q000008:administration & dosage*
chemical_list: D001897:Boronic Acids; D007166:Immunosuppressive Agents; D011719:Pyrazines; D014750:Vincristine; D000069286:Bortezomib
keywords: Evans syndrome; bortezomib; cord blood; pediatric hematology/oncology; stem cell transplantation; vincristine