RESUMEN
RESUMO Paciente feminina, 70 anos, apresentou-se com queixas de prurido ocular à esquerda, lacrimejamento e hiperemia há 2 meses. Ao exame físico, apenas hiperemia conjuntival discreta. Foi solicitada biópsia, que sugeriu a hipótese de pseudolinfoma pela insuficiência de critérios classificatórios. Após um ano a paciente retornou com os mesmos sintomas e proptose ocular à esquerda, associado a proliferação de aspecto linfático em conjuntiva bulbar, com presença de vasos sanguíneos, ocupando toda região superior e maior parte da medial, se estendendo até o limbo, sem oclusão do eixo visual e mobilidade ocular extrínseca preservada. Nova biópsia e imuno-histoquímica, foram compatíveis com linfoma de células B da zona marginal. Foram realizadas tomografias computadorizadas (TC) de crânio, pescoço e órbitas, revelando aumento do volume do músculo reto superior esquerdo, com intensa impregnação pelo contraste, acometendo ventre e tendão, com borramento de gordura adjacente. Foram programados 6 ciclos de quimioterapia com ciclofosfamida, doxorrubicina, vincristina e prednisona, CHOP, a cada 21 dias. Houve melhora dos sintomas oculares após o primeiro ciclo. Após o quarto, nova TC de órbitas evidenciou regressão do espessamento do músculo reto superior esquerdo, ainda melhor na TC realizada após o sexto ciclo. Os linfomas MALT são responsáveis por 5-17% de todos os casos de LNH, respondem por mais de 90% dos linfomas que acometem os anexos oculares, mas podem se originar em diversos tecidos. Devido a raridade da doença, carecem estudos prospectivos randomizados que definam um consenso terapêutico. A literatura sugere que o tratamento deve ser individualizado.
ABSTRACT Female patient, 70 years old, presented with complaints of left eye pruritus, tearing and redness, for 2 months. Physical examination revealed only mild conjunctival hyperemia. Biopsy was ordered, and suggested the hypothesis pseudo lymphoma by insufficient classification criteria.After a year, the patient returned with the same symptoms, and left ocular proptosis associated with lymphatic aspect of proliferation in bulbar conjunctiva, with the presence of blood vessels, occupying the entire upper region and most of the medial, extending into the limbo without occlusion the visual axis, extrinsic ocular motility preserved.Another biopsy and immunohistochemistry were compatible with a B cell marginal zone lymphoma. Tomographic studies of cranium, neck and orbits were performed, and the orbit showed enlargement of the left superior rectus muscle volume, with intense contrast uptake, affecting belly and tendon and blurring of adjacent fat. Six cycles of chemotherapy with cyclophosphamide, doxorubicin, vincristine and prednisone, CHOP, were done every 21 days. There was improvement in ocular symptoms after the first cycle. After the fourth, new scans from the orbits revealed regression of thickening of the left superior rectus muscle, and even better after the sixth cycle. The MALT lymphomas account for 5-17% of all cases of NHL, accounting for over 90% of lymphomas affecting the eye attachments, but can originate in different tissues. Due to the rarity of the disease, there are no randomized prospective studies to define a therapeutic consensus. The literature suggests that treatment should be individualized.
RESUMEN
Antecedentes. Los linfomas oculares (LO) y de los anexos oculares (LAO) tienen una incidencia de 7- 8% de todos los tumores extraganglionares. Objetivo. Describir las características clínicas, morfológicas e inmunofenotípicas de los LO y LAO atendidos en el Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán. Material y métodos. Se revisaron los expedientes de los pacientes con diagnóstico de LO y LAO de julio de 1994 a julio del 2005. Se analizaron los siguientes datos: presentación clínica, tratamiento, respuesta al tratamiento, supervivencia global, y supervivencia libre de enfermedad (SVLE). Resultados. Se analizaron 10 pacientes, 8 mujeres y 2 hombres. La media de edad fue de 50 años. La remisión completa (RC) se presentó en 8 de 10 pacientes y en los 6 pacientes con linfoma de tejido linfoide asociado a mucosas (MALT). Se encontraron dos con enfermedad refractaria, los cuales estaban en estadio IV. Conclusiones. En este estudio los LO y LAO correspondieron a 0.02% de todos los linfomas no Hodgkin (LNH) estudiados (498 casos). Los linfomas tipo MALT se presentan a edad más avanzada, se encontraron en estadios más tempranos y en todos hubo RC, con una SVLE promedio de 868 días y una media de supervivencia de 442 días.
BACKGROUND: Ocular lymphomas and ocular adnexae lymphomas (OL and OAL) constitute 7-8% of all extranodal lymphomas. OBJECTIVE: Describe the clinical, morphologic and immunophenotypic characteristics of OAL seen in our hospital. MATERIAL AND METHODS: Retrospective analysis of patient records with OL and OAL between July 1994 and July 2005. The following data was analyzed: Clinical presentation, therapy, treatment response, overall survival and disease free survival. RESULTS: Ten patients with OL and OAL were identified. Of these, 8 were women and 2 men. Median age was 50. Eight of 10 patients achieved complete remission, 6 of the 6 presenting MALT Lymphoma. Two patients with stage IV had refractory disease. CONCLUSIONS: In our series 0.02% of lymphomas were OL and OAL of a total 498 LNH. MALT lymphomas appear at a more advanced age, sixty percent of the cases were MALT lymphomas and were diagnosed during their early stages. Patients were followed during 21 months, global survival was 100%, free illness survival had a mean of 868 days and a survival median of 442 days.