Ocular manifestations as first signs of systemic T cell lymphoma in two cases

A 19-year-old Chinese female presented in Oct 2005 with fever, fatigue, cough and sputum. Accessory examination revealed positive serum Epstein-Barr virus (EBV) IgG/VCA and IgM/VCA, abnormal hepatic function and erythrocyte sedimentation rate. She was diagnosed as chronic active hepatitis and her symptoms relieved a lot after antibiotics and liver protection treatment. In Dec 2006, she complained of blurred vision and red eye OU, tinnitus and hearing loss of the right ear, as well as rhinobyon. Visual acuity was 20/100, Jr2 OD and 20/250, Jr1, OS, and best corrected visual acuity (BCVA) was not detected at this time. There were mixed congestion, diffused mutton-fat KPs, and anterior chamber cells and flare in both eyes (Fig. 1a, b). Fundus examination was not clear, but normal (Fig. 1c, d). B-scan ultrasonography showed slight vitreous opacities, OU. The diagnosis was “granulomatous uveitis OU”. Anterior uveitis did not respond well to topical 1% Pred Forte, so systemic corticosteroid and azathioprine were given. There were some improvement of anterior segment inflammation after treatment and BCVA improved to 18/20 OD and 20/20 OS at one time. But anterior segment inflammation was persistent and repeated, and cataract developed gradually. Fundus fluorescein angiography (FFA) was performed on Feb 9, 2007, there was high fluorescence of optic disk OU, no obvious fluorescein leakage of the vessels and no macular edema (Fig. 2). In May 2007, she complained of feet pain, lower extremity weakness and intermittent twitch. ENT consultation found a mass on the left side of pharynx. Final diagnosis was pharynx T cell non-Hodgkin’s lymphoma with CD3 (+) and CD56 (−). PCR detected TCR gene rearrangement. Cerebrospinal fluid examination also found massive naive cells. After chemotherapy of CHOP (CHOP = cyclophosphamide, epirulbicin hydrochloride, vindesine sulfate, prednisone) and COID (COID = methotrexate, vindesine sulfate, ifosfamide, dexamethasone) for 13 cycles, fever and feet pain relieved a lot. Phaco and IOL implantation was done for both eyes. Visual acuity improved to 20/16 OD and 20/12.5 OS. So far, her condition is stable for more than 10 years and there is no sign of disease progression.

A 37-year-old Chinese male presented on April 18, 2013, complaining blurred vision OU and recurrent abdominal pain for over a month. In March, he felt middle and upper abdominal pain, with abdominal distension, nausea, fatigue and profuse sweating. A few days later, he began to feel blurred vision OD, with water ripple feeling. He was diagnosed as necrotizing pancreatitis in local hospital. Abdominal pain relieved after treatment, but visual acuity of both eyes decreased. When the patient was referred to PUMCH, visual acuity was 20/125 OD and 20/32 OS. There were conjunctiva edema and congestion, gray-white KPs, anterior chamber cells and flare, and hazy vitreous OU. Fundus examination showed retina edema in the posterior area, retinal venous engorgement, and epiretinal membrane on the disk OU. The margin of optic disk was not clear OD (Fig. 3a, c). OCT showed serous detachment of the macular and retinal neuroepithelium edema OU (Fig. 3b, d). After using topical 1% Pred Forte and tropicamide phenylephrine eye drops for 2 weeks, anterior uveitis didn’t release. From April 28, the patient complained intermittent abdominal pain and fever. Serum amylopsin, lipase, transaminase and bilirubin were elevated. CT showed diffused enlargement of pancreas, edema of duodenal wall, thickening of gall blander wall. B ultrasound showed enlargement of the common bile duct. Visual acuity continued to decrease. On May 10, an emergency exploratory laparotomy operation was done. Unfortunately, the patient died of multiple organ failure 4 days later. Final histopathological diagnosis was gallbladder type II enteropathy-associated T-cell lymphoma (EATL).