Primary vitreoretinal lymphoma (PVRL) is a rare and aggressive high-grade non-Hodgkin lymphoma. PVRL always with non-specific symptoms and has therefore been called as masquerade syndrome. Thus, the early and correct diagnosis of PVRL is a difficulty. For PVRL, the imaging findings can be specific but variety, histological diagnosis is still the gold standard. Its diagnosis needs to be combined with clinical manifestations, imaging features, pathological diagnosis and molecular biology. With the advancement of technology, especially in the field of the cytokines detection and the gene expression profiling research, molecular biology diagnosis of PVRL is becoming a research hotspot and an important auxiliary diagnostic method.
Objective To analyze the results of diagnostic pars plana vitrectomy (PPV) in patients with uveitis of unknown cause. Methods This is a retrospective case series study. Sixty-five patients (67 eyes) with uveitis of unknown cause were enrolled in this study. There were 31 males (32 eyes) and 34 females (35 eyes). The ages were from 6 to 84 years, with the mean age of (55.00±18.56) years. All eyes were received PPV. Examination of vitreous samples consisted of microbial stains and culture, microbial DNA and antibody detection, cytokine measurement, cytology, flow cytometry and gene rearrangement detection. Results Vitreous analysis was positive in 40 of 67 eyes (59.7%). Positive results indicated bacterial endophthalmitis in 20 of 40 eyes (50.0%), lymphoma in 11 eyes (27.5%), viral IgM and IgG increased significantly in 3 eyes (7.5%), fungal endophthalmitis in 3 eyes (7.5%), IgG of toxocara increased significantly in 2 eyes (5.0%), IgG of toxoplasma Gondii increased significantly in 1 eye (2.5%). Conclusion The diagnostic yield of vitreous samples in uveitis eyes of unknown cause is 59.7%.
Objective To observe the ocular features and analyze the cause of misdiagnosis of intraocular nonHodgkin's lymphoma (IONHL). Methods A retrospective study was performed in 6 patients and related literatures were reviewed. The age of those patients was 46 to 68 yrs with an average of 562 yrs. Four cases were female, two cases were male. Five patients were unilateral and one case was bilateral case. All patients were investigated by Goldmann tonometer, fundus fluorescein angiography (FFA) and ophthalmic Bscan. Pathological samples come from enucleation and vitrectomy, and were analyzed histologically and immunohistochemically. Results Ophthalmological signs of IONHL included decrease visual acuity, red eye, aqueous flare; keratic precipitates (Kp), vitreous opacity, retinal detachment and multiple whiteyellow retinal lesions of different sizes and fuzzy boundaries. IONHL patients were always misdiagnosed as diverse and nonspecific symptoms and signs, two cases were misdiagnosed as retinitis or chorioiditis, one case as intermediate uveitis, one case as secondary glaucoma in uveitis. The duration from onset of symptom and sign to a final diagnosis was from 6 months to 24 months with an average of 13.3 months. Conclusions The clinical manifestation of IONHL was diverse and non-specific，and this disease was unknown to lots of doctors and easily be misdiagnosed.
Objective To investigate the relevance of primary pulmonary non-Hodgkin’s lymphoma ( PPNHL) imaging with pathology features, so as to improve the diagnostic accuracy. Methods Twenty-two patients of PPNHL were scanned by chest computed tomography, and the results of clinical and pathology features were analyzed. Results The resulting pathologic examination showed that all patients in 12 cases of low-grade lymphoma had MALT lymphoma( 54. 5% ) , histologically with slightly atypical small lymphocyte proliferated. The radiological findings indicated unilateral peripheral lesion which extended along mucous membrane in 11 patients ( 91. 7% ) and multilateral lesion in only 1 patients ( 8. 3% ) . CT features were illdefined nodule or mass with halo sign ( n =8, 66. 7%) , patchy infiltrate ( n = 4, 33. 3% ) , and ill-defined consolidations with air-bronchograms ( n =7, 58. 3% ) . The resulting pathologic examination showed that 10 patients were high and middle-grade lymphoma ( 45. 5% ) . The radiological findings indicated unilateral lesion in4 patients ( 40%) and multilateral lesion in 6 patients( 60% ) . CT features were ill-defined noduleor mass ( n =4, 40% ) , patchy infiltrate ( n = 1, 10% ) , mixed manifestation ( n =5, 50% ) , pleural effusion ( n =5, 50% ) , hilar and mediastinal lymph node enlargement ( n =2, 20% ) , atelectasis ( n =3, 30%) , and pulmonary interstitial with interlobular septal thickening ( n=2, 20% ) . Conclusions The imaging features of PPNHL rely on its’ basic pathology. Low-grade lymphoma is characterized by peripheral focal consolidation with air-bronchograms. High and middle-grade lymphoma is characterized by mixed manifestation of interstitial change and nodular focus. Analyzing CT features seriously may be helpful for diagnosis of PPNHL.
ObjectiveTo investigate the clinical, ophthalmological and pathological features of primary uveal lymphoma.MethodsRetrospective clinical study. From 2012 to 2018 in Beijing Tongren Eye Cener, 4 cases and 4 eyes of patients with primary uveal lymphoma were included in the study. Among them, 3 cases were male and 1 case was female. The average age was (54 ± 13.58) years old. The average time from initial diagnosis to pathological diagnosis was (18.50 ± 9.29) months. 3 cases were enucleated and 1 case was biopsied. Extranodal marginal zone lymphoma (EMZL) of the mucosa associated lymphoid tissue (MALT) was confirmed by pathological examination. BCVA, fundus color photography, color Doppler ultrasound and orbital MRI were performed in all eyes. UBM, OCT, FFA and ICGA were performed in 2 eyes, 3 eyes, 3 eyes and 2 eyes respectively. The clinical, imaging and pathological changes were observed. Following up time was ≥ 6 months.ResultsAt the initial diagnosis, BCVA was 0.6, 0.02 and 0.01 in 1, 2 and 1 eye respectively. Choroid, ciliary body and iris were involved in 3 eyes, choroid in 1 eye. The fundus of the eyes showed infiltration of choroid in yellow and white color, and the lesions were beyond the vascular arch to the equator and peripheral areas. Color Doppler ultrasonography showed that choroidal diffuse thickening and extrascleral extension (ESE) which was the corresponding hypoechoic areas behind the sclera. Among them, ESE showed crescent thickening in 1 eye and nodular thickening in 3 eyes. UBM showed that the echo of ciliary body was thicken and the internal echo was decreased with the iris involved. OCT showed that RPE was wavy and local retinal neuroepithelial layer detached. FFA showed that the early lesions were mottled with strong and weak fluorescence, and the late fluorescence leakage. The posterior wall of the eyeball was thickened and enhanced in MRI.ConclusionThe clinical manifestations of uveal lymphoma are various, color Doppler ultrasound has characteristic manifestations and ESE of crescent or nodular thickening is valuable in diagnosis.
ObjectiveTo observe the image characteristics of optical coherence tomography (OCT) in patients with primary vitreoretinal lymphoma (PVRL).MethodsA retrospective clinical study. Thirty-two eyes of 19 patients diagnosed with PVRL by vitreous pathology in the Department of Ophthalmology, Beijing Tongren Hospital from September 2016 to October 2019 were included in this study. There were 7 males and 12 females. The median age was 56 years. The mean time from symptom onset to final diagnosis was 6.1±3.8 months. The first diagnosis was uveitis in 12 cases (63.1%, 12/19), retinal vein occlusion in 2 cases (10.5%, 2/19), central retinal artery occlusion in 1 case (5.3%, 1/19), and suspected PVRL of camouflage syndrome in 4 cases (21.1%, 4/19). Routine ophthalmic examination and frequency-domain OCT examination were performed in all the patients, and typical images were stored for analysis. According to the examination results, PVRL OCT signs were divided into vitreous cells, inner retinal infiltration, outer retinal infiltration, retinal pigment epithelial (RPE) infiltration, sub-RPE infiltration, and subretinal fluid.ResultsVitreous cells were found in all eyes (100.0%, 32/32). RPE infiltrated were observed in 19 eyes (59.4%, 19/32), RPE infiltration in 16 eyes (50.0%, 16/32), outer retinal infiltration in 8 eyes (25.0%, 8/32), inner retinal infiltration in 16 eyes (50.0%, 16/32), and subretinal fluid in 4 eyes (12.5%, 4/32).ConclusionsPVRL OCT signs can involve vitreous and retinal anatomical levels, including vitreous cells, inner retinal infiltration, outer retinal infiltration, RPE infiltration, sub-RPE infiltration and subretinal fluid. The same patient can show multiple signs at the same time.
Primary vitreoretinal lymphoma (PVRL) is a rare type of non-Hodgkin's lymphoma with poor prognosis and the optimal treatment has yet to be determined. Its treatment has evolved from enucleation to ocular radiotherapy, systemic chemotherapy and intravitreal chemotherapy. Radiotherapy can effectively eradicate tumor cells but ocular recurrences are common. Systemic chemotherapy has become the mainstream option but there are problems with only-partial response of PVRL and high rate of recurrence. Intravitreal chemotherapy, primarily used as adjunctive to systemic chemotherapy, has achieved high remission rate and low rate of recurrence as well as with limited ocular complications. The tumor cells were cleared and the visual function preserved. However, issues about the drug applied, treatment protocols and goals of intravitreal chemotherapy, whether for visual preservation or survival improvement, are worthy for further study.
Epstein-Barr (EB) virus infection is associated with various tumors of lymphoid and epithelial origin. EB virus exists in most humans as a latent infection. EB virus latent infection-related genes play a key role in the EB virus latent infection, and also play an important role in promoting the occurrence and development of related tumors. This article will briefly introduce the characteristics of EB virus latent infection, the protein coding genes and non-coding genes related to EB virus latent infection (including EB virus nuclear antigen genes, EB virus latent membrane protein genes, EB virus encoded small RNA genes and EB virus microRNA genes), and the main functional mechanism of these EB virus latent infection-related genes in EB virus latent infection and subsequent tumorigenesis. The purpose is to providea theoretical basis for a comprehensive understanding of the EB virus latent infection and the mechanism of tumors caused by EB virus.
Objective To explore the clinical characteristics, diagnose and treatment of bronchiolitis obliterans (BO) associated with lymphoma and paraneoplastic pemphigus (PNP). Methods One patient with BO associated with lymphoma and paraneoplastic pemphigus was presented and literatures which searched through Wanfang Med online and PubMed on this subject were reviewed. Results A 44-year-old woman was admitted to this hospital because of oral ulcer for two years, and persistent dyspnea for fourteen months. She had been diagnosed with PNP one year earlier. Pulmonary function displayed severe obstructive ventilator dysfunction, negative response to the bronchodilator. Chest CT showed bronchiolectasis. The ratio of neutrophils increased in bronchoalveolar lavage fluid. The pathology of ultrasound-guided neck and inguinal lymph node biopsy showed non-Hodgkin’s lymphoma. Based on the clinical presentation, the image and pathology of this patient, the diagnosis of having PNP with BO secondary to the lymphoma was made. After 10 cycles of CHOP chemotherapy, lymphoma and PNP went into remission, but the patient died from respiratory failure. The literatures were reviewed and 15 patients were found, out of them 60% (9/15) were female, 47% (7/15) follicular lymphoma and 47% (7/15) diagnosis of clinical BO which did not be supported by pathology. Lymphoma-PNP-BO was a clinical syndrome and 67% of the initial presentations were PNP. Patients died within 7 to 60 months. Conclusions BO associated with lymphoma and PNP is rare and the most common initial presentation is PNP. BO is the major cause of death. Treatments included chemotherapy, corticosteroid and immunosuppressor, lymphoma and PNP can be alleviated, but BO is aggravating.
ObjectiveTo evaluate the safety and efficacy of the intravitreal methotrexate treatment in patients with primary vitreoretinal lymphoma (PVRL). MethodsRetrospective non-comparative interventional case series. Fourteen patients (26 eyes) with biopsy-proven PVRL were included in the study. All patients received examination of Snellen chart visual acuity, fundus color photography and optical coherence tomography (OCT). Among the 24 eyes with recordable visual acuity, 17 eyes has initial visual acuity≥0.1 (0.45±0.20) and 7 eyes with initial visual acuity ranged from light perception to hand movement. The vitreous opacities and (or) subretinal yellowish-white lesions and retinal pigment epitheliumuplift were observed in all eyes. All eyes were treated with intravitreal methotrexate (4000 μg/ml, 0.1 ml) injections according to a induction-consolidation-maintenance regimen. For 26 treated eyes, each received an average of (11.5±6.3) injections. Twenty eyes had finished theintraocular chemotherapy, while 6 eyes had not. Eight of 20 eyes were clinically confirmed free of tumor cells by diagnostic vitrectomy, 12 eyes were still with tumor cell involvement.The follow-up was ranged from 2 to 48 months, the mean time was 18 months. The examination of BCVA, fundus color photography and OCT were performed. No tumor cell was defined as clinical remission. Visual acuity was scored as improved or declined obviously (improved or declined 2 lines) or mild improved or declined (changed within 2 lines). ResultsTwenty eyes achieved clinical remission after (3.5±3.6) injections, 12 eyes of 20 eyes with tumor cell involvement before chemotherapy achieved clinical remission after (5.8±3.0) injections. The mean visual acuity of seventeen eyes with initial visual acuity 0.1 in induction phase and at the end of treatment were 0.36±0.23 and 0.56±0.20, respectively. Compared with before treatment, the visual acuity was mild declined in induction phase (t=1.541, P>0.05), but mild improved at the end of treatment (t=2.639, P<0.05). The visual acuity at the end of treatment in 7 eyes with initial visual acuity<0.1 was ranged from no light perception to 0.1. Of 14 patients, 2 patients have been fatal because of brain lesions progression at 42 and 48 months after diagnosis of primary central nervous system lymphoma. No ocular recurrence was noted during the follow-up in 20 eyes who finished intraocular chemotherapy. ConclusionsPVRL patients can achieve clinical remission after (3.5±3.6) injections by intravitreal chemotherapy of methotrexate, and the visual acuity improved mildly. No ocular recurrence was found during follow-up.