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A 34-year-old male patient, with a history of acute myeloid leukemia (AML) treated by bone marrow allograft seven years prior to presentation, was referred for painful decreased vision with redness of the left eye. Visual acuity was 20/20 in the right eye and limited to counting fingers in the left eye. Slit lamp examination of the left eye demonstrated episcleral vessels dilation, myotic pupil, corneal edema, hypopyon, rubeosis of the iris, and posterior synechiae (Image 1A). Fundus was not accessible. Polymerase chain reaction for the herpes-virus family and toxoplasmosis in the aqueous humor (AH) were negative. Sub-conjunctival injections of dexamethasone, systemic steroids, and antibiotics (ofloxacin 200mg bid and imipenem 500mg tid) did not bring any improvement. Repeated anterior chamber tap obtained a viscous yellowish AH and cytological analysis showed myeloblasts containing azurophilic granules and 1 or 2 Auer rods (Image 1B). Fluorescent in situ hybridization analysis of the cells in the AH demonstrated a MLL gene rearrangement in 84% of analyzed nuclei (Image 1C) that was similar to the one observed during the initial disease, seven years ago. Systemic work-up including a TEPscan and a bone marrow aspiration did not reveal any systemic relapse. Brain MRI and lumbar puncture did not show any infiltration of the CNS. The patient later developed severe intraocular hypertension requiring surgical iridectomy. Pathology of the iris showed myeloblasts (Image 1D). Ultrasound demonstrated thickening of the retina and optic disc swelling, suggestive of posterior infiltration. A systemic aracytin-based chemotherapy was started. On follow-up, hypopyon disappeared, visual acuity improved to 20/200 after cataract surgery, and repeated anterior chamber tap did not show any myeloblast. The patient was still in remission one year after the initiation of the treatment and 5 years after the beginning of his ocular relapse.

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Figure 1. Panel 1A: Clinical presentation of the patient on slit lamp examination. A: Anterior uveitis with hypopyon (arrow), (B) circumferential posterior synechiae (arrow) and cataract due to chronic intraocular inflammation, leading to an iris Bombay. C: Episcleral (plain arrow) and scleral vessels dilatation (discontinued arrow). D: Rubeosis of the iris (arrow). Panel 2: Histological and cytological analysis. A: Myeloblasts with one to two Auer rods in the AH obtained after anterior chamber tap in the hypopyon. (B–D). Magnification 20×. Pathology of the iris, obtained after iridectomy for the iris “Bombé”. A: Hematoxylin–Eosin staining (HES), demonstrating infiltration of the iris by the myeloblasts. B: Myeloperoxydase staining was positive on myelobalsts. C: CD34 staining of the iris: the myeloblasts were negative for CD34 staining. Panel 3: Fluorescent in situ hybridization (FISH) analysis on AH with the MLL dual color, break apart probe (Abbott). We observed a normal fusion signal (yellow arrow) and a splitted signal between 5′MLL (green arrows) and 3′MLL (red arrows) in 84% of analyzed nuclei, indicating a MLL gene rearrangement.

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Patients with uveitis, especially with a hypopyon, undergo an extensive work-up to determine whether it is part of a general disease. The most frequent causes of hypopyon include endophthalmitis, B27-related uveitis [1], and Behçet's disease [2]. Any severe uveitis, such as tuberculosis, sarcoidosis, or herpes virus-associated-uveitis can also present with a hypopyon. Rarely, it can reveal a malignancy (usually an intraocular lymphoma) and is referred to as masquerade syndrome.

Intraocular location of AML is extremely rare [3-5]. Leukemic hypopyon has mostly been reported with acute lymphoid leukemia [6]. Matano et al. have reviewed patients with AML and leukemic hypopyon. All had an extramedullary infiltration. Leukemic hypopyon was associated with a systemic relapse and a poor prognosis. All patients but one died within two years. Our patient had relapsing uveitis for at least five years at the time of diagnosis, and systemic work-up did not identify any medullary or extra-medullary infiltration. This is the first report of an isolated ocular location of AML without systemic involvement in an adult patient with long-term follow-up.

Anterior chamber tap is an easy procedure with few complications. It enables direct access to the intraocular media for cytological and molecular analysis. Prompt sampling of AH in refractory hypopyon can help to state the diagnosis.

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