ABSTRACT
The incidence of Epstein-Barr virus (EBV) viraemia and lymphoproliferative disease (LPD) was studied in a consecutive cohort of 128 paediatric patients undergoing stem cell transplantation (SCT) with reduced-intensity conditioning (RIC; n = 65) or conventional-intensity conditioning (CIC; n = 68). Following CIC, six of 68 (8%) developed viraemia; all remained asymptomatic. EBV viraemia (23 of 65 patients = 35%, P < 0.001) and LPD (10 of 65 = 15%, P < 0.001) were significantly more frequent following RIC. Of the 23 RIC patients who developed viraemia, eight remained asymptomatic, five had symptomatic viraemia (fever +/- rash), and 10 patients developed LPD, two of whom died. An absolute lymphocyte count of <0.3 x 10(9)/l at the time of onset of viraemia was strongly predictive of development of LPD (P < 0.05) in this group. The incidence of viraemia was significantly higher in patients receiving serotherapy with antithymocyte globulin (ATG; 15 of 43, 35%) than Campath (12 of 73, 16.4%, P < 0.05). Primary immunodeficiency and acute graft-versus-host disease were associated with EBV viraemia in univariate analysis, but were not independent risk factors. In conclusion, EBV viraemia and LPD appear to be significantly more common in children following RIC SCT, particularly with selective depletion of recipient T cells relative to B cells following the use of ATG. This probably reflects the profound immunosuppression following RIC SCT, together with the incomplete ablation of recipient-derived B cells.
Subject(s)
Epstein-Barr Virus Infections/complications , Herpesvirus 4, Human , Lymphoproliferative Disorders/surgery , Stem Cell Transplantation , Transplantation Conditioning/methods , Adolescent , Alemtuzumab , Antibodies, Monoclonal/therapeutic use , Antibodies, Monoclonal, Humanized , Antibodies, Neoplasm/therapeutic use , Antilymphocyte Serum/therapeutic use , B-Lymphocytes/immunology , Child , Child, Preschool , Epstein-Barr Virus Infections/immunology , Female , Graft vs Host Disease/immunology , Graft vs Host Disease/virology , Humans , Immunization, Passive , Incidence , Infant , Lymphocyte Count , Lymphoproliferative Disorders/immunology , Lymphoproliferative Disorders/virology , Male , Multivariate Analysis , Risk Factors , T-Lymphocytes/immunologyABSTRACT
Infantile myofibromatosis is the most common fibrous tumor of infancy. The generalized form of the disease is associated with a high rate of early mortality, especially if visceral structures are involved. Various therapeutic strategies have been used in these patients, including high-dose chemotherapy, with the risk of therapy-related toxicity. The authors present two cases of generalized infantile myofibromatosis, with life-threatening visceral and nonvisceral involvement, in which the patients were cured with a combination of low-dose chemotherapy and intensive care. The authors propose a prospective international trial using a safe low-dose chemotherapy protocol to test the efficacy of this treatment strategy.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Myofibromatosis/drug therapy , Soft Tissue Neoplasms/drug therapy , Combined Modality Therapy , Critical Care , Dactinomycin/administration & dosage , Female , Humans , Infant, Newborn , Male , Methotrexate/administration & dosage , Muscle Neoplasms/congenital , Muscle Neoplasms/drug therapy , Myofibromatosis/congenital , Osteolysis , Prednisolone/administration & dosage , Remission Induction , Respiration, Artificial , Skin Neoplasms/congenital , Skin Neoplasms/drug therapy , Soft Tissue Neoplasms/congenital , Vinblastine/administration & dosage , Vincristine/administration & dosageABSTRACT
Children who have completed treatment for acute lymphoblastic leukaemia (ALL) are commonly followed up for the first 5 years with regular full blood counts (FBCs) to monitor for relapse of disease. There is little evidence to suggest that this practice improves the detection rate of unexpected relapse. Surveillance FBCs, performed on 43 children with relapsed ALL between 1990 and 1999, were analysed. Of the 42 relapses in children off therapy, only two were detected by an abnormal FBC. Routine FBCs in asymptomatic children off therapy lacks specificity in detecting unexpected relapses and maybe safely discontinued.
