ABSTRACT
Bu, combined with TDM-guided dosing, is associated with fewer graft failures/relapses and lower toxicity in pediatric HSCT. We aimed this retrospective study for comparison of weight- and age-based dosing in terms of clinical outcomes such as time to engraftment, early complications, EFS, OS, and toxicity profiles in children receiving iv Bu. Sixty-one children who underwent HSCT from April 2010 to February 2013 by means of a Bu-based conditioning regimen and completed 100 days after transplantation at Ankara Children?s Hematology and Oncology Hospital Bone Marrow Transplantation Unit were enrolled in this study. SOS and neutropenic fever occurred more frequently in the weight-based dosing group. We found a statistically significant correlation between Bu dose and the incidence of SOS (r = 0.26, p = 0.04). Multivariate analysis showed only weight-based dosing of Bu was a significant predictor of SOS (HR = 9.46; p = 0.009). However, no relationship was found between two groups in terms of hemorrhagic cystitis, engraftment syndrome, acute or chronic GvHD, time to engraftment, chimerism, TRM, OS, and EFS rates. Weight-based dosing of Bu may cause higher incidence of SOS and early infectious complications at the places where TDM of Bu cannot be performed.
Subject(s)
Busulfan/administration & dosage , Hematopoietic Stem Cell Transplantation , Infusions, Intravenous , Adolescent , Age Factors , Body Weight , Child , Child, Preschool , Cyclophosphamide/therapeutic use , Female , Fever/etiology , Graft Survival , Graft vs Host Disease/etiology , Hematologic Diseases/therapy , Hematologic Neoplasms/therapy , Humans , Infant , Male , Multivariate Analysis , Neutropenia/etiology , Recurrence , Retrospective Studies , Transplantation Conditioning , Treatment OutcomeABSTRACT
The primary objective was to evaluate the response rate of rituximab therapy for children with chronic immune thrombocytopenic purpura (ITP) and Evans syndrome (ES) and immune reconstitution of these children after rituximab therapy. Eleven patients with chronic ITP and 2 with ES between 6 and 18 years of age and platelet count less than 20 × 10(9)/L received rituximab. Overall response (OR) was defined as an increase in platelet count above 50 × 10(9)/L. The mean age of 13 children (9 girls, 4 boys) was 11.2 ± 3.8 years (6-18). One of the patients with ES had been splenectomized; others were not. The patients mean follow-up time was 10.3 ± 9.3 months after rituximab therapy. Two patients achieved complete response, 4 patients achieved partial response, and OR rate was 46% (6 of 13) after therapy. Seven patients have no response. In conclusion, rituximab may be considered prior to splenectomy in children with chronic ITP and ES with an acceptable toxicity profile.
Subject(s)
Anemia, Hemolytic, Autoimmune/drug therapy , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Antineoplastic Agents/therapeutic use , Purpura, Thrombocytopenic, Idiopathic/drug therapy , Thrombocytopenia/drug therapy , Adolescent , Anemia, Hemolytic, Autoimmune/blood , Anemia, Hemolytic, Autoimmune/immunology , Antibodies, Monoclonal, Murine-Derived/adverse effects , Antineoplastic Agents/administration & dosage , Antineoplastic Agents/adverse effects , Child , Female , Hemostasis/drug effects , Humans , Platelet Count , Purpura, Thrombocytopenic, Idiopathic/blood , Purpura, Thrombocytopenic, Idiopathic/immunology , Rituximab , Thrombocytopenia/blood , Thrombocytopenia/immunology , Treatment OutcomeABSTRACT
Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder of the immune system that is associated with frequent involvement of the central nervous system (CNS). The MR imaging and CT findings of the CNS infiltration have been reported in the past; however, the diffusion-weighted imaging (DWI) findings have not been previously described. We present MR imaging findings in a case of secondary HLH with CNS involvement, with an emphasis on the DWI findings.
