title: Impact of thrombotic thrombocytopenic purpura on leukemic children undergoing bone marrow transplantation.
abstract: Thrombotic thrombocytopenic purpura (TTP) has emerged as one of the main transplant-related complications over the last 15 years. The current study defines the incidence and the risk factors for the occurrence of TTP in 131 consecutive leukemic children who were transplanted between January 1994 and December 1997 at four Italian pediatric centers. Patients with ALL (101), AML (21), MDS (9), underwent an HLA-identical sibling BMT (82) or an HLA-identical unrelated BMT (49), receiving a conditioning regimen consisting of high-dose chemotherapy in 24 patients and of F-TBI combined with high-dose chemotherapy in 107 patients. The diagnosis of TTP was retrospectively evaluated on the basis of parallel criteria. TTP treatment varied according to the protocol of each treatment center. Twenty-eight of 131 patients (21.4%) developed TTP at a median of 46 days (range 21-80) after BMT. Multivariate analysis demonstrated that the risk of TTP was higher in patients who underwent unrelated BMT (P value = 0.02). Acute GVHD, stage of disease at BMT, conditioning with TBI, gender, age, did not appear to be associated with the occurrence of TTP. As to the outcome, TTP resolved in 19 patients while in nine it was the principal cause of death (32.1%). In patients with TTP, LDH peak value was the only statistically significant factor (P = 0.001) related to severe TTP. In conclusion, our experience demonstrates that leukemic children undergoing BMT, especially from an unrelated donor, should be carefully assessed for TTP which appears to be a severe and relatively common transplant-related complication when strict diagnostic criteria are applied.
authors: Clinica Pediatrica Ospedale S Gerardo di Monza, Università degli Studi di Milano Bicocca, Italy.
authors: C
authors: C
authors: Uderzo
authors: M
authors: M
authors: Fumagalli
authors: P
authors: P
authors: De Lorenzo
authors: A
authors: A
authors: Busca
authors: E
authors: E
authors: Vassallo
authors: S
authors: S
authors: Bonanomi
authors: E
authors: E
authors: Lanino
authors: G
authors: G
authors: Dini
authors: S
authors: S
authors: Varotto
authors: C
authors: C
authors: Messina
authors: R
authors: R
authors: Miniero
authors: M G
authors: MG
authors: Valsecchi
authors: A
authors: A
authors: Balduzzi
mesh_terms: D000208:Acute Disease; D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D005343:Fibrinolytic Agents / Q000627:therapeutic use; D006086:Graft vs Host Disease; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015994:Incidence; D007223:Infant; D007770:L-Lactate Dehydrogenase / Q000097:blood; D007938:Leukemia / Q000150:complications / Q000628:therapy*; D008297:Male; D010949:Plasma; D010956:Plasmapheresis; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use; D011089:Polydeoxyribonucleotides / Q000627:therapeutic use; D011379:Prognosis; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood / Q000453:epidemiology / Q000209:etiology* / Q000628:therapy; D012074:Remission Induction; D012189:Retrospective Studies; D012307:Risk Factors; D019172:Transplantation Conditioning; D016896:Treatment Outcome
chemical_list: D005343:Fibrinolytic Agents; D007166:Immunosuppressive Agents; D010975:Platelet Aggregation Inhibitors; D011089:Polydeoxyribonucleotides; C036901:defibrotide; D007770:L-Lactate Dehydrogenase