ABSTRACT
Between 1995 and 2003, seven cases of posttransplant lymphoproliferative disorder (PTLD) were identified among 1,116 patients who received allogeneic hematopoietic stem cell transplantations (HSCT) at Catholic HSCT Center (overall incidence 0.6%). Five (71.4%) patients had episodes of acute graft-versus-host-disease (GVHD) and were treated with steroids. Cervical lymphadenopathy was observed in most cases (71.4%), but clinical symptoms varied depending on the involved sites. Pathologic findings varied: 1 case of plasmacytic hyperplasia, 3 of polymorphic PTLD, 2 of diffuse large B-cell lymphoma, 1 of large T-cell lymphoma, which proved to be associated with Epstein-Barr virus (EBV). The proportion of EBV-negative PTLD was 33.3%. Five patients demonstrated a good response to treatment (treatment response rate 71.4%). The overall mortality was 42.8%, and one death was directly attributable to PTLD. The incidence of PTLD is expected to increase, based on the rising use of grafts from alternative donors and recent clinical features of PTLD manifested by a disseminated and fulminant nature. It is necessary to have a high level of suspicion when monitoring patients and readily adopt prompt and effective cellular immunotherapy for PTLD.
Subject(s)
Middle Aged , Male , Humans , Female , Adult , Adolescent , Transplantation, Homologous , Lymphoproliferative Disorders/etiology , Lymphoma, T-Cell/etiology , Lymphoma, Large B-Cell, Diffuse/etiology , Lymphoma, Large B-Cell, Diffuse/etiology , Lymphoma, B-Cell/etiology , Korea , Hematopoietic Stem Cell Transplantation/adverse effects , Epstein-Barr Virus Infections/etiologyABSTRACT
Se describe un paciente masculino de 10 años de edad con historia de mialgia, debilidad progresiva en miembros inferiores, fiebre y vómitos. Se postuló el diagnóstico clínico de Síndrome de Guillain-Barré el cual fue subsecuentemente demostrado con el resultado de líquido cefalorraquídeo (LCR) y electromiografía. A su admisión se describió una faringoamigdalitis exudativa y una sinusitis etmoidal, y posteriormente durante el curso de su enfermedad desarrolló una parálisis facial derecha. No habia historia de enfermedad diarreica aguada o enfermedad respiratoria previa al inicio de sus síntomas neurológicos. Los frotis y cultivos de heces fueron negativos por Campylobacter jejuni, al igual que la inmunofluorescencia para virus respiratorios y serología por CMV. Se documentó mediante una IgM positiva en suero la infección aguda por el virus Epstein-Barr. El paciente fue tratado exitosamente con un curso de cinco días de gammaglobulina endovenosa y un mes después, en la consulta externa, se documenta la ausencia de secuelas neurológicas