RESUMEN
Dendritic cell leukemia (DCL) or hematodermic tumor is an uncommon subtype of acute leukemia. In contrast to adult cases, children tend to have a less aggressive course. The diagnosis of DCL should be considered when its characteristic morphologic features are present and leukemic cells co-express CD4 and CD56. Cases of DCL among pediatric patients have been reported to respond to therapeutic regimens for acute lymphoblastic leukemia, but details regarding the specifics of therapy are lacking.
Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Células Dendríticas/patología , Leucemia/tratamiento farmacológico , Leucemia/fisiopatología , Niño , Preescolar , Femenino , Humanos , MasculinoRESUMEN
PURPOSE: To describe a patient with conjunctival posttransplantation lymphoproliferative disorder (PTLD). DESIGN: Interventional case report. METHODS: A 8-year-old boy, three months status post bone marrow transplantation with bilateral enlarged gelatinous bulbar conjunctiva was examined. Biopsy of the bulbar conjunctiva was performed. RESULTS: Conjunctival biopsy revealed a polymorphous infiltrate of lymphoid cells with large atypical immunoblastic lymphoma cells, plasmacytoid lymphocytes, and plasma cells. B-cell markers CD20 and CD79a were positive. Plasma cells exhibited restriction for kappa immunoglobulin light chain and were positive for CD79a. Most cells were positive for Epstein-Barr virus (EBV) encoded ribonucleic acid. EBV-related polymorphic PTLD was diagnosed and treated with discontinuation of cyclosporine, reduction in prednisone dosage and administration of EBV-specific cytotoxic T lymphocytes. The conjunctival lesions resolved during the next five weeks. CONCLUSIONS: Posttransplantation lymphoproliferative disorder rarely involves the ocular structures with most cases presenting as a masquerade syndrome. This case illustrates that PTLD can involve the conjunctiva and further expands the clinical spectrum of ocular PTLD.