Gaucher D, Bodaghi B, Charlotte F, Schneider C, Cassoux N, Lemaitre C, Leblond V, Rao N, LeHoang P
Service d'Ophtalmologie, Groupe Hospitalier Pitié-Salpêtrière, 4783 boulevard de l'Hôpital, 756 Paris cedex 13, France.
J Fr Ophtalmol. 2005 Jan;28(1):31-8. doi: 10.1016/s0181-5512(05)81022-8.
Diagnostic and therapeutic management of masquerade syndromes associated with MALT-type lymphoma.
We report the cases of three patients referred for acute or chronic severe loss of visual acuity. All of them suffered from uveitis or scleritis resistant to corticoid treatment. Each one had posterior uveitis associated with retinochoroidal infiltration, anterior scleritis associated with uveal effusion syndrome, or bilateral pan-uveitis associated with serous retinal detachment. Two patients had medical records of systemic malignancies. They underwent an extensive work-up. Search for an infectious, immune or malignant disease was negative but orbital imaging showed a posterior scleral thickening in all cases.
Diagnosis of mucosa-associated lymphoid tissue (MALT) ocular lymphoma was assessed in all cases after scleral biopsy. Immunohistochemical analysis showed CD20(+) and CD5(-) scleral lymphocyte infiltrates. The three patients underwent chemotherapy associated with radiotherapy. Lesions were stabilized in the first case, whereas six lines of visual acuity improvement was noted in the second one, and full recovery was achieved after treatment in the third case.
Clinical manifestations of this ocular lesion seem to be multiple, masquerading as different types of posterior uveitis or scleritis. In the three cases, we did found other systemic locations of MALT-type lymphoma. Diagnosis was mainly based on pathological analysis. Fast visual recovery in two cases demonstrated the importance of an early diagnosis and a specific treatment. All cases of chronic and corticoid-resistant posterior uveitis require a specialized radiological evaluation followed, if necessary, by multiple orbital biopsies.
与黏膜相关淋巴组织（MALT）型淋巴瘤相关的伪装综合征的诊断和治疗管理。
我们报告了3例因急性或慢性严重视力丧失前来就诊的患者。他们均患有对皮质类固醇治疗耐药的葡萄膜炎或巩膜炎。每例患者分别患有伴有视网膜脉络膜浸润的后葡萄膜炎、伴有葡萄膜渗漏综合征的前巩膜炎或伴有浆液性视网膜脱离的双侧全葡萄膜炎。2例患者有全身恶性肿瘤病史。他们接受了全面检查。感染性、免疫性或恶性疾病检查均为阴性，但眼眶影像学检查显示所有病例均有后巩膜增厚。
所有病例经巩膜活检后均确诊为黏膜相关淋巴组织（MALT）眼淋巴瘤。免疫组织化学分析显示巩膜淋巴细胞浸润为CD20（+）和CD5（-）。3例患者接受了化疗联合放疗。第1例患者病变稳定，第2例患者视力提高了6行，第3例患者治疗后完全恢复。
这种眼部病变的临床表现似乎多种多样，可伪装成不同类型的后葡萄膜炎或巩膜炎。在这3例病例中，我们确实发现了MALT型淋巴瘤的其他全身部位。诊断主要基于病理分析。2例患者视力快速恢复证明了早期诊断和特异性治疗的重要性。所有慢性和皮质类固醇耐药的后葡萄膜炎病例都需要进行专门的影像学评估，必要时进行多次眼眶活检。
