Intravascular B-Cell Lymphoma (Angiotropic Lymphoma) With Choroidal Involvement

Intravascular large B-cell lymphoma is a rare form of extranodal lymphoma characterized by the presence of large lymphoma cells in the lumen of small blood vessels, involving multiple organs. The diagnosis is usually made at the time of autopsy. We report a patient with intravascular large B-cell lymphoma who had vision loss from choroidal involvement, which improved with systemic therapy.

A 44-year-old white man was initially seen with bilateral blurred vision, Coombs-negative hemolytic anemia, and a maculopapular skin rash on both upper arms. The patient's best-corrected visual acuity (Snellen) was OD 20/40 −2 and OS 20/50. There were bilateral serous detachments involving the macula (Figure 1A). A small, superficial intraretinal hemorrhage was noted along the inferotemporal vascular arcade in the right eye, and peripheral flame-shaped intraretinal hemorrhages were present in both eyes. Fluorescein and indocyanine green angiography demonstrated irregular filling of the choroid early with bilateral pinpoint areas of fluorescence later (1 day) (Figure 1C). B-scan ultrasonography showed thickening of the choroid bilaterally (Figure 1B).

The patient had a normocytic anemia with an increased reticulocyte count, lactate dehydrogenase level, and bilirubin concentration. A computed tomographic scan of his chest and abdomen showed hazy ground-glass infiltrates throughout the lungs (Figure 2A), splenomegaly, and diffuse edema within the subcutaneous tissues of the thorax and abdomen. A biopsy specimen of his skin lesions showed nonspecific inflammatory cell infiltration of the dermis. A bone marrow biopsy specimen revealed hypercellularity with trilineage hyperplasia consistent with a peripheral destructive process. The blood smear demonstrated several atypical lymphocytes with convoluted nuclei. Cerebrospinal fluid examination re sults were normal, while a lung biopsy specimen revealed large, atypical CD20-positive and CD3-negative lymphocytes filling the lumen of the capillaries in the alveolar septae (Figure 2B and C). These findings were diagnostic of malignant intravascular lymphoma of B-cell phenotype (intravascular lymphomatosis).

A regimen of hyper-CVAD (cycle 1: cyclophosphamide, vincristine, doxorubicin, and dexamethasone) chemotherapy, intrathecal methotrexate and cytarabine, and methylprednisolone was initiated 10 days after initial examination. A week later, there was a marked improvement in visual acuity (20/20 OU) and resolution of serous detachments and choroidal thickening, shown by repeat fluorescein angiography and indocyanine green angiography (Figure 1E and F). The patient continued to do well 2 months after starting therapy.

Intravascular large B-cell lymphoma is a rare form of extranodal malignant lymphoma in which the lymphoid cells are principally found within small vessels without extensive involvement of bone marrow or lymphoid tissue.¹ Aberrant expression or deletion of certain cell adhesion molecules critical for lymphocyte trafficking and transvascular migration (CD29 and CD54) contributes to the tumor's intravascular and disseminated pattern of distribution.² The tumor expresses CD20 and other B-cell markers and does not express the T-cell marker CD3.³ At diagnosis, the lymphoma typically involves various organs, including the central nervous system, skin, lung, kidneys, spleen, and adrenal glands.⁴ Other types of lymphoma may separately coexist with intravascular large B-cell lymphoma in the same patient.² Hematologic abnormalities, like microangiopathic and autoimmune hemolytic anemia, as in our case, are often associated with this disease process.¹ The prognosis is generally very poor because of the usually delayed diagnosis. Intravascular large B-cell lymphoma can also involve the small vessels of the choroid. The case presented herein adds the eye as another end organ involved by this rare malignancy.

Correspondence: Dr Pulido, Mayo Clinic, Department of Ophthalmology, 200 First St SW, Rochester, MN 55901-0001 (pulido.jose@mayo.edu).

Author Contributions: Dr Pulido, principal investigator, had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.

Financial Disclosure: None reported.

Funding/Support: This project was funded by an unrestricted grant from Research to Prevent Blindness, New York, NY.