RESUMEN
Lymphomatoid granulomatosis (LG) is a rare systemic vasculitis caused by Epstein Barr virus induced transformation of the B-cells in a T-cell rich environment. The predominant clinical presentations are confined to the pulmonary system however; extra-pulmonary manifestations can sometimes be the main feature of the disease. Here in we describe a 52-year-old female who presented with symmetric polyarthritis and generalized stiffness for 7 months and papular lesions over extremities for 3 months duration. She in addition had generalized lymphadenopathy. Histopathological examination of the cutaneous lesions confirmed LG. Patient died despite therapy with cyclophosphamide and prednisolone. This is the first report of LG mimicking rheumatoid arthritis from India.
Asunto(s)
Artritis/diagnóstico , Artritis Reumatoide/diagnóstico , Diagnóstico Diferencial , Femenino , Humanos , Granulomatosis Linfomatoide/complicaciones , Persona de Mediana EdadRESUMEN
A case of lymphomatoid granulomatosis in a previously healthy 13-year-old Thai girl presenting with right sixth cranial nerve palsy and severe upper airway obstruction was reported. Cranial nerve palsy later disappeared spontaneously but the patient developed multiple pulmonary nodules and cavity leading to pulmonary insufficiency. Her course was complicated with septicemia which limited the use of corticosteroid and cytotoxic drugs. She finally expired with pseudomonas sepsis in addition to pulmonary and liver involvement. This is the first case of lymphomatoid granulomatosis in a child ever reported in Thailand. Lymphomatoid granulomatosis should be included in the differential diagnosis of upper airway obstruction with pulmonary nodules and cavity and multi-organ involvement in children.
Asunto(s)
Adolescente , Factores de Edad , Obstrucción de las Vías Aéreas/etiología , Femenino , Humanos , Enfermedades Pulmonares/complicaciones , Granulomatosis Linfomatoide/complicaciones , Tomografía Computarizada por Rayos XRESUMEN
Se presenta el caso de un paciente con insuficiencia respiratoria aguda y lesiones cutáneas, cuyas biopsias mostraron una granulomatosis linfomatoide en pulmones y piel y una médula ósea con hiperplasia granulocítica y megacariocítica. A diferencia de la mayoría de los casos publicados, éste tuvo un curso inicial muy agudo, las placas de tórax mostraron un infiltrado reticulonodular difuso e histológicamente, además de las células linfoides atípicas, se observaron polimorfonucleares con leucocitoclasia. Se discuten conceptos en relación a las lesiones linfoproliferativas angiocéntricas y se concluye que todas éstas, son muy probablemente linfomas T desde un inicio. La producción de linfocinas por las células neoplásicas podría explicar la mezcla de elementos reactivos, la necrosis y la hiperplasia de médula ósea