Name

Céphalée et photopsies: premières manifestations cliniques d'un lymphome intravasculaire à grandes cellules B

To report the case of a large B-cell intravascular lymphoma with ocular involvement.

The case of a large B-cell intravascular lymphoma is reported in order to note the importance of neuro-ophthalmological symptoms as initial manifestations of systemic or neurological diseases.

A 36-year-old female presented to our clinic with cephalea and photopsia. Best-corrected visual acuity was 8/10 in both eyes. Slit-lamp examination was unremarkable and fundoscopy showed solely a small haemorrhage in the left eye. Optical Coherence Tomography was performed and revealed a mild macular oedema in her right eye. Fluorescein Angiography was also performed showing mild leakage in the right macular area and a fluorescence pattern in peripheral retina that resembles occlusive vasculitis in both eyes. Cranial Tomography and Magnetic Resonance Imaging exhibited patched hypodense lesions in the white subcortical substance of frontal location with nonspecific etiology, so a Positron Emission Tomography was carried out with pathological uptake of the cervix, which was biopsied with the result of a large B-cell intravascular lymphoma. Extension studies were carried out, and the disease was staged as IV-B lymphoma with infiltration of the liver, spleen, bone marrow with hemophagocytic syndrome and cerebral vasculitis. The patient underwent chemotherapy R-CHOP both systemic and intrathecal and today she remains asymptomatic and continues the chemotherapy treatment with an adequate response. 

Intravascular large B-cell lymphoma is an uncommon aggressive lymphoma characterised by a growth of large B-cells in the lumen of all sized blood vessels. It presents with many nonspecific symptoms such as fever and may involve the nervous system. A hemophagocytic syndrome with hepatosplenic involvement and cytopenia may be associated and skin involvement has been also described in some variants. The most common chemotherapy regime includes rituximab which has improved the prognosis since its introduction. The disease can present relapses after treatment, so it is necessary to pay attention to the symptoms, especially neurological manifestations.

Intravascular large B-cell lymphoma is an aggressive tumour which may have neuro-ophthalmological manifestations at the beginning. An adequate exploration can reveal unclear signs and leads to a proper diagnosis which could improve the prognosis of the disease. Once again we would like to note the importance of ophthalmic exploration and collaboration with other specialists in the correct study of neuro-ophthalmological symptoms often overlooked.