Pseudo-inflammatory manifestations of choroidal lymphoma resembling Vogt-Koyanagi-Harada disease: case report based on multimodal imaging

Abstract

Background Hematologic malignancies occasionally cause serous retinal detachment (SRD); however, its pathogenesis remains unclear. Here we present the imaging characteristics of metastatic choroidal lymphoma masquerading as Vogt-Koyanagi-Harada (VKH) disease. Case presentation A 45-year-old Japanese woman was referred to our clinic because of bilateral SRD with blurred vision. Fluorescein angiography revealed multiple pinpoint leakage followed by pooling OU. Enhanced depth imaging optical coherence tomography showed marked choroidal thickening OU. Laser speckle flowgraphy detected choroidal circulation impairment OU. Although these results totally agreed with the inflammatory manifestations of acute VKH disease, indocyanine green angiography demonstrated various sizes of sharply marginated hypofluorescent lesions that seemed atypical for the finding of VKH disease, i.e., vaguely marginated hypofluorescent small dots. Cerebrospinal fluid pleocytosis was not detected. Blood tests revealed leukocytosis together with elevation of lactate dehydrogenase and soluble interleukin-2 receptor levels. Corticosteroid pulse therapy did not improve any ocular findings. Bone marrow biopsy was then performed, leading to a definite diagnosis of diffuse large B-cell lymphoma. After starting systemic chemotherapy, both SRD and choroidal thickening resolved rapidly with visual recovery. However, choroidal hypoperfusion persisted, which contrasted distinctly with the inflammatory pattern of VKH disease, i.e., the restoration of choroidal blood flow in parallel with normalization of choroidal thickness. Conclusions Our detailed multimodal observations highlighted the differential imaging features of choroidal lymphoma despite close resemblance to VKH disease especially at the initial stage. Impaired circulation in the thickened choroid marked the pseudo-inflammatory pathogenesis of SRD due to choroidal involvement with neoplastic, but not inflammatory cells.

Value of Combined Diagnosis for Choroidal Lymphoma: A Case Report

“…However, a stark difference was observed in our case, i.e., high spot fluorescence around the optic disc at the early stage, without any obvious fluorescence leakage, indicating milder damage to the pigment epithelium than that in VKH disease. The typical ICGA manifestations included low fluorescence dots of uniform size in the middle stage of VKH disease [ 26 ], which are different from those of our case. In our patient, ICGA showed persistent low fluorescence spots or flakes of different sizes in the middle and late stages, consistent with the findings of Fukutsu et al [ 2 ].…”

“…Moreover, patients with metastatic intraocular lymphoma often require systemic chemotherapy [ 25 ]. The prompt improvement in SRD and choroidal thickening after chemotherapy confirmed choroidal involvement in diffuse large B-cell lymphoma, the chief cause of metastatic CL [ 26 ].…”

Laser speckle flowgraphy findings in focal scleral nodule

“…In the FSN lesion, the choroidal circulation may be locally impaired given previously reported hypofluorescence on indocyanine green angiography (ICGA) and nonperfusion of choriocapillaris on optical coherence tomography angiography [1]. To date, surveying macular choroidal blood flow using laser speckle flowgraphy (LSFG) has played an important role in elucidating the pathophysiology of various fundus diseases [2][3][4][5][6][7][8]; however, the LSFG findings of FSN has not been reported so far. Here, we report the LSFG findings in 2 FSN cases.…”

Systemic lymphoma masquerading as Vogt-Koyanagi-Harada syndrome: Report of a case with multimodal imaging and histopathology

“… 13 While these features are not incompatible with a diagnosis of VKH, they should prompt careful consideration of other diagnostic possibilities, including leukemia and lymphoma, which can present similarly. 14 , 15 , 16 , 17 , 18 , 19 The initial response to high dose systemic corticosteroid in this case, followed by a refractory and worsening course despite continued high dose systemic corticosteroid and immunosuppression, should alert the clinician that this is likely a masquerade syndrome, and further work-up, including systemic imaging to consider malignancy, should be strongly considered. A unique aspect of this case is the absence of other inflammatory signs reported in other cases, such as vitritis, retinitis, intraretinal or subretinal infiltration, or vitreous hemorrhage, suggesting that systemic lymphoma can present with exudative retinal detachments as the only ocular manifestation.…”