MALT lymphoma of the lacrimal apparatus (mucosa-associated lymphoid tissue)

Mucosa-associated lymphoid tissue lymphoma can occur in various localizations. We describe the case of a patient presenting bilateral MALT lymphoma involving a lacrimal gland and a lacrimal duct. Biopsy specimens are required for histologic diagnosis. Such low-grade lymphomas are generally treated medically with radiotherapy or chemotherapy. Prognosis is generally favorable. A complete work-up is required to determine extension. These patients should be followed regularly.     

Atypical conjunctival CD5 positive mucosa-associated lymphoid tissue (MALT) lymphoma

A 61-year-old woman with intermittent ocular irritation redness and swelling of her eyes was followed up and investigated. The diagnosis was conjunctival CD5 positive mucosa-associated lymphoid tissue (MALT) lymphoma, which is an atypical and rare type of pathology of the eye, in which the clinical course/behavior is not fully understood. Our experience showed that this disease is indolent and responds well to radiotherapy. It can recur in the fellow eye, even after as many as eight years of successful treatment with no evidence of recurrences in the previously affected eye. Therefore, long-term periodic follow-up is mandatory to detect recurrences in such patients. Further studies are also required to elucidate a more conclusive clinical behavior of this disease so that a clearer therapeutic and management plan can be devised for future patients with this disease.     

Localized conjunctival mucosa-associated lymphoid tissue (MALT) lymphoma is amenable to local chemotherapy

Purpose Conjunctival mucosa-associated lymphoid tissue (MALT) lymphoma is a rare, low-grade, non-Hodgkin’s B-cell lymphoma. We report our successful management of localized conjunctival MALT lymphoma with topical Mitomycin C (MMC). Methods This is a case report. A 35-year-old woman had a mobile painless 1.5 × 1-cm mass in the left conjunctiva for 2 years. Examination revealed two similar masses in the right conjunctiva. Incisional biopsy for immunohistochemical stain and PCR of the left conjunctival mass showed MALT lymphoma. She was given four courses of topical 0.04% MMC eyedrops. There was transient conjunctival injection and superficial punctate keratopathy which responded to topical steroid and lubricant. Results The lesions regressed completely after the fourth cycle of treatment and repeat biopsy confirmed complete remission. Conclusion This is the first report of localized conjunctival MALT lymphoma being successfully treated by topical MMC with minimal local controllable side effects.     

Subconjunctival mucosa-associated lymphoid tissue (MALT) lymphoma arising in Tenon’s capsule

Purpose We report the first case of subconjunctival mucosa-associated lymphoid tissue (MALT) lymphoma arising in Tenon’s capsule (fascia bulbi). Methods A 75-year-old woman presented with painless swelling of the superior bulbar conjunctiva in her left eye. During the biopsy of the bulbar lymphoid lesion, it was noticed that the conjunctiva was movable and that the lesion was located in the subconjunctiva. The tissues were studied by conventional light microscopy, immunohistochemistry, flow cytometry, and gene rearrangement analysis. Results Histopathological examination revealed that a diffuse lymphoid infiltrate consisting of small-sized lymphoid cells was present in Tenon’s capsule but not in the substantia propria of the conjunctiva. Immunohistochemical and flow cytometric studies documented tumor cells of B-lymphocyte lineage. Molecular analysis demonstrated positive immunoglobulin heavy chain gene rearrangement. The final diagnosis was subconjunctival MALT lymphoma arising in Tenon’s capsule. Conclusion Ophthalmologists and pathologists need to distinguish the subconjunctival lymphoma that arises in Tenon’s capsule from the conjunctival lymphoma in the substantia propria during diagnosis of epibulbar lymphoid tumors. This material is not under consideration for presentation and has not been previously presented. The authors received no specific financial support for this study and have no financial interest in any company or product discussed in it.     

Mucosa-associated lymphoid tissue lymphoma of the lacrimal gland.

PURPOSE: Mucosa-associated lymphoid tissue lymphoma recently has been defined as a distinct subtype of non-Hodgkin's lymphoma with characteristic clinicopathologic features. A 37-year-old woman with systemic lupus erythematosus and unilateral mucosa-associated lymphoid tissue lymphoma of the lacrimal gland is described. METHODS: The tumor was totally excised by lateral orbitotomy. Immunohistochemical studies were performed with UCHL-1, CD20 (L26), leukocyte common antigen, cytokeratin (CAM5), and kappa and lambda light chain antibodies. RESULTS: The tumor was composed of centrocyte-like lymphocytes, cells with plasmacytoid features, and lymphoepithelial lesions. Most of the cells expressed the CD20 protein and were positive for immunoglobulin kappa light chain. The patient received no supplemental therapy. No systemic dissemination or local recurrence occurred during a follow-up of 26 months. CONCLUSION: The features of this case support the association between systemic diseases and the subsequent development of mucosa-associated lymphoid tissue lymphoma.     

Combination of adult inclusion conjunctivitis and mucosa-associated lymphoid tissue (MALT) lymphoma in a young adult

PURPOSE: To report a patient who was diagnosed with combined adult inclusion conjunctivitis (AIC) and mucosa-associated lymphoid tissue (MALT) lymphoma. METHODS: This is a case report. RESULTS: An 18-year-old male patient presented with chronic conjunctivitis and giant follicles. Evaluation by chlamydial antigen assay was positive. Conjunctival biopsy for the immunohistochemical stain and polymerase chain reaction of the left eye showed MALT lymphoma. CONCLUSIONS: MALT lymphoma can masquerade as other ocular surface diseases. Chlamydial infection causes chronic inflammation of the conjunctiva. Both of these diseases should be considered as a differential diagnosis of refractory follicular conjunctivitis. It is worthy of further study to determine whether chronic inflammation resulting from chlamydial infection increases the risk of MALT lymphoma or it is coincidental.     

Radiotherapy for localized orbital mucosa-associated lymphoid tissue lymphoma

Fourteen patients with localized orbital mucosa-associated lymphoid tissue lymphoma diagnosed between 1998 and 2005 were reviewed. Five patients were males and 9 were females, with a mean age of 58 years. In 8 patients, the disease arose from the conjunctiva, and in 6 patients, it originated from the retrobulbar space. Patients were treated with radiotherapy alone at a dose range from 30 to 54 Gy. All patients with conjunctival lymphoma achieved complete remission (CR). Four patients with retrobulbar lymphoma obtained CR, and unconfirmed CR (CRu) was observed in 2 cases. Orbital extraconal lymphoma disappeared. However, intraconal cord-like tumor and mass involving the medial extraocular muscle remained with the absence of regrowth over the long term. There have been very few reports discussing the histology of residual mass after radiotherapy. Residual mass was suggested to be reactive lymphoid hyperplasia. As a dose of more than 40 Gy induced dry eye syndrome or cataract, the dose must not exceed 40 Gy in order to achieve safe treatment of orbital mucosa-associated lymphoid tissue lymphoma.     

Ocular adnexal mucosa-associated lymphoid tissue lymphoma with polyclonal hypergammaglobulinemia

PURPOSE: To determine the characteristics of patients with primary ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma associated with polyclonal hypergammaglobulinemia. DESIGN: Case series study. METHODS: Among 81 Japanese patients with primary ocular adnexal MALT lymphoma, seven patients (9%) were diagnosed with polyclonal hypergammaglobulinemia. Patient clinical data included a history of autoimmune disease and dissemination. Peripheral blood collected from all patients was analyzed for serum levels of rheumatoid factor, soluble interleukin-2 receptor (sIL-2R), and immunoglobulins at the time of diagnosis and after each treatment. RESULTS: Seven patients with polyclonal hypergammaglobulinemia had elevated serum levels of rheumatoid factor, sIL-2R, immunoglobulin G (IgG), and immunoglobulin E (IgE) at the time of diagnosis. One patient had Sjogren syndrome. Six patients (86%) had a dissemination of the MALT lymphoma or lymphadenopathy at the time of diagnosis. Histopathologic examination of the patients with lymphadenopathy revealed not only MALT lymphoma but also secondary follicles. None of the seven patients showed improvement in serum levels of IgG, rheumatoid factor, or sIL-2R in spite of complete regression of the ocular lesions after radiotherapy. After administration of cyclophosphamide/doxorubicin/vincristine/prednisone and/or rituximab to three patients, all three showed improved serum levels of IgG, rheumatoid factor, and sIL-2R. CONCLUSIONS: Patients with ocular adnexal MALT lymphoma and polyclonal hypergammaglobulinemia have elevated serum levels of rheumatoid factor, sIL-2R, and IgE, and high dissemination or lymphadenopathy. These unique characteristics may correlate with the systemic immunologic imbalances.     

Bilateral conjunctival mucosa-associated lymphoid tissue lymphoma misdiagnosed as allergic conjunctivitis

PURPOSE: This report aimed to discuss two nearly identical cases of primary conjunctival mucosa-associated lymphoid tissue (MALT) lymphoma mimicking allergic conjunctivitis. METHODS: Two patients were referred for intractability to conventional treatment of initial diagnoses of allergic conjunctivitis. On ocular examination, normally pigmented, giant papilla-like lesions were found bilaterally in the upper conjunctiva. Excisional biopsies were performed. RESULTS: Histopathologic and immunohistochemical examination of the conjunctival biopsies indicated MALT lymphoma in both patients. The patients subsequently received radiation therapy and achieved complete remission with no evidence of recurrence during follow-up periods of 13 and 11 months. CONCLUSION: Conjunctival low-grade lymphoma may share similar clinical features with allergic conjunctivitis. Ophthalmologists should be concerned that primary conjunctival low-grade malignant lymphoma can be misdiagnosed as allergic conjunctivitis     

眼附属器黏膜相关淋巴组织淋巴瘤的研究进展

黏膜相关淋巴组织（MALT）淋巴瘤是非霍奇金淋巴瘤中结外边缘带淋巴瘤（MZL）的一种特殊类型。眼附属器MALT淋巴瘤是眼眶恶性肿瘤中较常见的一种，可发生在结膜、泪腺、眼睑和眼眶。近年来，国外许多学者在MALT淋巴瘤的临床特点、病理形态学、免疫表型、分子遗传学及分子生物学方面对它的发生机制在基因水平有了新的认识。就国外对眼附属器MALT淋巴瘤的分子遗传学异常在肿瘤细胞内部信号转导通路中作用的研究做一综述。     

眼附属器黏膜相关淋巴组织淋巴瘤的病因学研究进展

越来越多的证据表明感染因素与眼附属器黏膜相关淋巴组织(mucosa-associated lymphoid tissue,MALT)淋巴瘤的发生发展有关,但对于具体的病因还存在争议,目前的研究主要集中在幽门螺杆菌、衣原体、丙肝等方面,现就对该问题作一综述。     

眼附属器MALT淋巴瘤的临床特征及复发因素分析

目的 通过对眼附属器MALT淋巴瘤的临床特征和复发因素的研究,以期对本病有一个全面的认识和了解,减少临床误诊、漏诊和复发,提高早期诊断率及生存率.方法 回顾性分析1997年1月至2010年6月第二军医大学附属长征医院眼科收治的79例病理确诊为IE期眼附属器MALT淋巴瘤患者的临床表现、影像学结果、治疗方式及预后情况.结果 79例病例中,原发于眼眶49例,结膜14例,泪腺10例,眼睑6例.发生在眼眶内的MALT淋巴瘤多呈包绕眼环的不规则生长,无明显边界,临床表现最为常见的是眼球突出、眼球运动受限.而发生在结膜的MALT淋巴瘤多位于穹窿部结膜,表现为结膜充血、肿块呈“鲑鱼肉”样外观.治疗方法主要包括手术及放射治疗.本组病例全部行手术治疗,全部切除58例,局部切除21例.所有患者均在手术后2～4周行眼局部放射治疗.放疗半年后,有15例出现复发.患者双眼发病(P =0.0143)和对放疗的初始反应(P＜0.0001)与肿瘤的复发有相关性;而性别、年龄、发病部位、肿瘤大小及手术方式等因素对复发均无影响.结论 发生在眼眶内的MALT淋巴瘤多呈包绕眼环的不规则生长,无明显边界.患者双眼发病和对放疗的初始反应与肿瘤的复发有相关性,而手术方式等因素对复发均无影响.选择合适的手术方式可降低术后并发症的发生.     

Detection of Chlamydia pneumoniae in a bilateral orbital mucosa-associated lymphoid tissue lymphoma

PURPOSE: To detect Chlamydia pneumoniae (C. pneumoniae) gene in a patient with bilateral orbital musoca-associated lymphoid tissue (MALT) lymphoma. DESIGN: Interventional case report. METHODS: A 47-year-old Chinese man with recurrent bilateral orbital masses underwent surgical biopsy. Ophthalmologic and radiographic examinations, routine histology, immunohistochemistry, and molecular analysis for immunoglobulin heavy chain (IgH), bcl-2/IgH gene translocation, and Chlamydia genes were performed. RESULTS: Pathology revealed orbital MALT lymphoma with B-cell monoclonality. In addition to IgH gene rearrangement, C. pneumoniae DNA was detected in the lymphoma. CONCLUSIONS: The finding of C. pneumoniae molecular signatures in this case suggests a possible association of Chlamydia and orbital MALT lymphoma. The infection may contribute to the development of the lymphoma.     

眼附属器MALT淋巴瘤的临床与病理学特征分析

目的:探讨眼附属器包括眼睑、眼眶、泪腺等部位黏膜相关淋巴组织淋巴瘤(lymphphoma of mucosa-associated lymph-oid tissue,MALT)的临床、病理学特征与治疗方法。方法:分析24例眼附属器MALT淋巴瘤的临床、病理学及免疫组化结果,以及手术后治疗与预后情况。结果:24例眼附属器MALT淋巴瘤患者经影像学检查后均行手术治疗,其中16例行放疗,2例行化疗。20例随访3mo～7a后均存活。结论:影像学检查可辅助诊断眼附属器MALT淋巴瘤、术后病理活检及免疫组化分型检查可确诊本病,眼结膜MALT淋巴瘤预后较好,伴全身淋巴瘤转移术后辅以化疗或放疗。     

眼附属器MALT淋巴瘤的临床分析

目的 探讨眼附属器包括眼睑、结膜，眼眶和泪腺等部位MALT淋巴瘤的临床诊断要点，影像学检查特征与治疗和预后。方法 回顾性分析1994年至2005年在第二军医大学长征医院眼科确诊的45例眼附属器MALT淋巴瘤的临床表现、影像学检查、手术治疗及病理结果与预后情况。结果 眼附属器MALT淋巴瘤23例发生于眼眶，10例发生于眼睑，8例发生于泪腺，4例发生于结膜。B超、CT、MRI均能进行定位、定性诊断，B超能较好地显示病变内部结构和形态；CT对眶骨结构和肿瘤侵犯范围与周围组织的关系显示良好，具有定性诊断价值；MRI对软组织的分辨力强，成像参数根据不同的信号强度可提示病变的内部结构。45例均行手术治疗；其中22例辅以放疗，10例结合化疗。42例随访4～135个月，1例于手术切除后25个月转移至肺死亡，其余均存活。结论 眼附属器MALT淋巴瘤好发于眼眶上方及眼睑，影像学检查对MALT淋巴瘤的诊断及鉴别诊断有帮助。手术切除肿瘤，结合病理诊断和临床分期判断能提示较准确的病情估计，病变可长期局限，预后较好，一般不会出现全身转移和向弥漫性大细胞高度恶性淋巴瘤转化。     

Immunophenotypic profiles for distinguishing orbital mucosa-associated lymphoid tissue lymphoma from benign lymphoproliferative tumors

Purpose

To evaluate novel immunophenotypic profiles of patients with orbital lymphoproliferative tumors.

Methods

From 2006 to 2014, surgical biopsies from consecutive patients with newly diagnosed orbital mucosa-associated lymphoid tissue (MALT) lymphoma and IgG4-related ophthalmic disease (IgG4-ROD) were collected from Tokyo Medical University Hospital. A total of 44 tumors from 44 patients were analyzed, including 21 with orbital MALT lymphoma (11 men and 10 women, mean age 67.8 ± 13.4 years) and 23 with definitive IgG4-ROD (9 men and 14 women, mean age 60.5 ± 15.1 years). Patients with secondary orbital MALT lymphoma and MALT lymphoma with IgG4-ROD were excluded. All patients were immunocompetent Asian adults. Samples were analyzed by immunohistochemistry and flow cytometric analysis. Flow cytometry was performed with the following antibodies: CD3, CD4, CD5, CD8, CD10, CD19, CD20, CD23, CD25, CD30, CD34, and CD56 lambda and kappa chains.

Results

Expression of CD25 and CD19 was higher in patients with orbital MALT lymphoma compared to patients with IgG4-ROD (p < 0.001). In contrast, expression of CD3, CD4, and CD23 was higher in patients with orbital IgG4-ROD compared to those with MALT lymphoma (all p < 0.01). The presence of CD23 and CD25 in the specimens was confirmed by immunohistochemistry.

Conclusion

The present study elucidated the novel immunophenotypic features of orbital MALT and benign lymphoproliferative disorders such as IgG4-ROD. Among them, CD23 and CD25 have shown a disease-specific expression pattern.

     

Spectral-domain optical coherence tomography of conjunctival mucosa-associated lymphoid tissue lymphoma with presumed choroidal involvement

Background  

Conjunctival mucosa-associated lymphoid tissue (MALT) lymphoma has been well-described, but rarely do these lesions demonstrate intraocular involvement. We report a case of conjunctival MALT lymphoma with intraocular involvement and novel spectral-domain ocular coherence tomography (SD-OCT) findings.     

Local chemotherapy with interferon-alpha for conjunctival mucosa-associated lymphoid tissue lymphoma: a preliminary report

OBJECTIVE: To evaluate the efficacy of subconjunctival interferon-alpha for the treatment of conjunctival mucosa-associated lymphoid tissue (MALT) lymphoma. DESIGN: Small, noncomparative, interventional case series. PARTICIPANTS: Five patients with histologically proven conjunctival MALT lymphoma were studied prospectively. METHODS: Patients were given 1,500,000 international units (IU) of interferon-alpha (IFN-alpha) (Roferon-A) subconjunctivally inside the lesion, three times a week for four weeks. If there was even a minimal response, a further cycle of 1,000,000 IU three times a week for four weeks was administered. Patients received a maintenance dose of 1 million IU, every 15 days for 4 times, after clinical resolution of the lesion. MAIN OUTCOME MEASURES: Patients were followed clinically, with slit lamp examination, for evidence of tumor disappearance or recurrence. RESULTS: Complete response was obtained in all patients. The lesion resolved completely by the eighth week. Four patients did not show any local recurrence with a median follow-up of 21 months (12-36 months). One patient presented with a recurrence after 11 months, in association with systemic lymphoma progression. CONCLUSIONS: Local chemotherapy with IFN-alpha seems to be an effective treatment modality, alternative to radiotherapy, for conjunctival MALT lymphomas.