RESUMO
INTRODUCTION: Anaplastic large-cell lymphoma (ALCL) is an infrequent childhood malignancy whose diagnosis and treatment have largely evolved since its initial description in 1985. PATIENTS AND METHODS: We retrospectively reviewed our experience in the field, and report here a single institution experience focusing on diagnostic and therapeutic milestones achieved as novel tools have been developed. This is a series of 9 children diagnosed from 1987 to 2007. RESULTS: Our first patient was diagnosed shortly alter this entity was described based on morphology and Ki-1 positivity, while the diagnostic work-up for the last two children included accurate molecular diagnosis for ALK-NPM rearrangement. Despite a wide variety of multimodal therapies used over time, only one patient died of toxicity during progression and another child relapsed and survived alter an autograft. After 156 months of median follow-up (range 4-245), 8 out of 9 children are alive, free of disease. CONCLUSIONS: Our series exemplifies the long journey travelled from the definition of a new entity only 20 years ago to the molecular characterization not only with diagnostic but also therapeutic purposes. Besides this, significant efforts are being made to recruit all European patients into a multinational collaborative trial in order to start drawing major evidence-based conclusions.
Assuntos
Linfoma Anaplásico de Células Grandes/diagnóstico , Linfoma Anaplásico de Células Grandes/tratamento farmacológico , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Criança , Pré-Escolar , Feminino , Humanos , Linfoma Anaplásico de Células Grandes/mortalidade , Masculino , Estudos Retrospectivos , Resultado do TratamentoRESUMO
INTRODUCTION: Posttransplant lymphoproliferative disorder (PTLD) constitutes a heterogeneous group of diseases. We summarize the experience of our hospital, one of Spain's largest series of renal (294), liver (47) and allogeneic stem cell transplants (67), where four cases of PTLD have developed related to complex viral infections. METHODS: Case 1 was a 24-month-old boy diagnosed with acute lymphoblastic leukemia who underwent allogeneic stem-cell transplantation (SCT). He was seropositive for Epstein-Barr virus (EBV) and developed an aggressive Bcell non-Hodgkin's lymphoma (B-NHL) related to EBV reactivation and human herpesvirus 6 (HHV-6) infection. Cases 2, 3, and 4 developed after kidney transplantation and were all EBV seronegative. Case 2 had associated cytomegalovirus (CMV) and EBV infection. Cases 3 and 4 only revealed EBV viral load. Cases 1, 3, and 4 progressed rapidly, with fatal outcome. Global incidence of PTLD in our series is 1.1%. CONCLUSION: PTLD is a rare but life-threatening condition. Although EBV plays a clear role in its pathogenesis, other associated viral infections could trigger this situation. Current therapies include rituximab, decreasing immunosuppressive drugs. and conventional chemotherapy.