Anaplastic Large Cell Lymphoma Involving Anterior Segment of the Eye
This case report followed the tenets of the Declaration of Helsinki and was approved by the institutional review board of Dongguk University, Ilsan Hospital.\nA 36-year-old woman presented with multiple facial masses in the left submandibular and forehead area (). Incisional biopsy of the forehead lesion revealed ALCL with positivity for CD30, CD3, and ALK (). No evidence of other body part involvement was found on magnetic resonance imaging (MRI), positron emission tomography (PET), or cerebrospinal fluid analysis. With a diagnosis of ALCL involving skin only, the patient received 1 cycle of CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisolone) chemotherapy. On day 5 after chemotherapy, the patient complained of conjunctival injection, ocular pain, and blurred vision in the right eye. Ophthalmic examination detected a mild inflammatory reaction in the anterior chamber with normal visual acuity, intraocular pressure (11 mmHg), and fundus findings in the right eye. Topical fluorometholone (0.1%) was prescribed to control inflammation. However, the symptoms rapidly aggravated. On the second visit after 2 days, the patient's right eye demonstrated severe inflammatory reaction with dense fibrin clots around the pupillary margin in the anterior chamber with significant corneal edema (). No fundus details were observed and ocular ultrasonography revealed a relatively clear posterior segment (). Because the complete blood count showed reduced white blood cell count of 1.61 × 103/µL (normal range, 4.0 to 10 × 103/µL) with an absolute neutrophil count of 580 (normal range, 1,500 to 7,700), the presumed diagnosis was endogenous endophthalmitis or neoplastic masquerade syndrome. Cytologic examination and aqueous humor culture were simultaneously conducted with vancomycin (1 mg/0.1 mL), ceftazidime (2 mg/0.1 mL), and dexamethasone (250 µg/0.1 mL) intravitreal injections. A 0.5% moxifloxacin ophthalmic solution was applied every two hours. Vancomycin was administered in travenously. A cytospin smear of aqueous humor aspiration revealed atypical lymphocytes (). On the next day of aqueous humor culture, acute angle closure developed (intraocular pressure rose to 40 mmHg) due to pupillary block by dense fibrin (). Tissue plasminogen activator (25 µg/0.05 mL) was injected into the anterior chamber to reduce fibrin and open the aqueous passage, but this therapy failed. The increased intraocular pressure (IOP) (over 40 mmHg) was uncontrollable despite maximum tolerated drug therapy including intravenous mannitol, anti-glaucoma ophthalmic solutions, and oral acetazolamide. Blood and aqueous humor cultures showed no bacterial growth. Laser iridotomy was attempted to relieve the pupillary block, but fibrin materials closed the opening within one day. Surgical removal of dense fibrotic membrane was attempted but failed due to recurrent bleeding and fibrotic membranes. MRI showed focal enhancement of the iris, ciliary body, and anterior chamber without posterior segment involvement (). A PET scan revealed increased fluorodeoxyglucose (FDG) metabolism in the right eye, right thyroid, and uterine wall (). Oncologists recommended localized radiation therapy to the right orbit. After 2 consecutive radiation therapy sessions with 3 Gy each, intraocular pressure normalized to 19 mmHg, the anterior chamber deepened, and fibrin material gradually disappeared. After 30 Gy of radiation therapy in total, the patient's best-corrected visual acuity was restored to 20 / 25 with an IOP of 11 mmHg without glaucoma medication (). No evidence of tumor recurrence was detected at 18 months after radiation therapy.