RESUMEN
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.
Asunto(s)
Trasplante de Médula Ósea/efectos adversos , Enfermedades de la Conjuntiva/etiología , Infecciones por Virus de Epstein-Barr/etiología , Infecciones Virales del Ojo/etiología , Trastornos Linfoproliferativos/etiología , Biomarcadores/metabolismo , Niño , Enfermedades de la Conjuntiva/diagnóstico , Enfermedades de la Conjuntiva/metabolismo , Ciclosporina/administración & dosificación , ADN Viral/sangre , Infecciones por Virus de Epstein-Barr/diagnóstico , Infecciones por Virus de Epstein-Barr/metabolismo , Infecciones Virales del Ojo/diagnóstico , Infecciones Virales del Ojo/metabolismo , Humanos , Técnicas para Inmunoenzimas , Hibridación in Situ , Trastornos Linfoproliferativos/diagnóstico , Trastornos Linfoproliferativos/metabolismo , Masculino , Prednisona/administración & dosificación , Linfocitos T Citotóxicos/inmunología , Síndrome de Wiskott-Aldrich/cirugíaRESUMEN
PURPOSE: To characterize the clinical and imaging features of orbital leukemic tumors in 27 patients seen and treated at St. Jude Children's Research Hospital. METHODS: A retrospective review was performed on the clinical and imaging records of 27 consecutive patients with a diagnosis of orbital leukemic tumors. The following data were extracted from the patients' records: age at diagnosis of orbital leukemic tumors, sex, race, national origin, type of leukemia, temporal relationship of orbital disease to diagnosis of leukemia, survival from diagnosis of orbital leukemic tumor, laterality of the orbital disease, location of the mass within the orbit, imaging features of the mass, chemotherapeutic protocol, treatment with bone marrow transplant, and orbital radiation. RESULTS: The median age at diagnosis of orbital leukemic tumor was 8 years (range, 1-18 years). Nineteen of the 27 patients were male, and 21 patients were born and lived in the United States. Twenty-one patients had acute myeloid leukemia, five had acute lymphoblastic leukemia, and one had chronic myelogenous leukemia. In 85% of patients (n = 23), the diagnosis of leukemia was based on the bone marrow examination findings. Orbital imaging revealed homogenous masses that molded to one or more orbital walls without bony destruction. Nine patients had bilateral orbital involvement. All patients received multiagent systemic chemotherapy, and 14 underwent bone marrow transplantation. Five patients received external beam radiation for the treatment of orbital disease. Fifteen (55.6%) of the 27 patients were alive at the time of the study. The median survival for all patients was 4.75 years (range, 0.1-24 years) after the diagnosis of orbital disease. CONCLUSIONS: Orbital leukemic tumors occur most commonly in the first decade of life, in association with acute myeloid leukemia. They appear as homogenous masses along the orbital walls. Although the overall survival rate for patients with leukemia has improved over the past 3 decades, the mortality of patients who develop orbital leukemic tumors remains high.