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Unilateral hypopyon in a child as a first and sole presentation in relapsing acute lymphoblastic leukemia

Wadhwa, Neeraj MD, FRCS; Vohra, Rajpal MD; Shrey, Dinesh MD; Iyer, V K MD; Garg, Satpal MD

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Indian Journal of Ophthalmology: May–Jun 2007 - Volume 55 - Issue 3 - p 223-224
doi: 10.4103/0301-4738.31947
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Abstract

Most types of leukemias may have protean ocular manifestations ranging from leukemic retinopathy to involvement of the iris and anterior chamber.1 Anterior chamber involvement in cases of acute lymphoblastic leukemia (ALL) relapse is typically bilateral.2 We report a child suffering from ALL in remission presenting with unilateral leukemic hypopyon uveitis as an initial and sole sign of ALL relapse.

Case Report

A four-year-old male child, with history of ALL since one and a half years of age, presented with a two-month history of pain, redness, watering and photophobia in the right eye (RE) more than left. Patient was declared to be in remission after the last cycle of chemotherapy received six months back. Suspecting a relapse, bone marrow biopsy, total leucocyte count, differential leucocyte count and peripheral blood smear was done at presentation and found to be normal. On examination, patient was photophobic and not cooperative for visual acuity testing. Examination of RE demonstrated severe conjunctival injection along with dirty white hypopyon and severe reaction in the anterior chamber [Fig. 1].

Figure 1
Figure 1:
Slit-lamp photograph of the right eye under diffuse illumination showing dirty white-colored hypopyon with conjunctival and ciliary congestion

Left eye was quiet and had a normal looking disc, retina and retinal vasculature. The rest of the systemic examination was normal.

Topical treatment with corticosteroid and atropine ointment along with systemic steroid 1 mg/kg body weight was started. The hypopyon did not resolve even after two weeks of treatment. Anterior chamber paracentesis was done to characterize the disease. Fluid aspirated from the anterior chamber was mixed with 1ml of polysol solution and two cytospin slides were prepared immediately. One of these was air-dried and stained with May Grunwald Giemsa stain. The other was fixed in 95% alcohol and subjected to Papanicolaou stain. On microscopic examination, both slides showed large numbers of blast cells [Fig. 2] consistent with infiltration of the anterior chamber by acute lymphoblastic leukemia. The patient was referred back to the oncology department for further treatment where he underwent cranial radiation to treat ocular relapse and bone marrow transplantation after systemic relapse was also confirmed with repeat bone marrow biopsy.

Figure 2
Figure 2:
Photomicrograph of cytospin slides showing numerous leukemic blasts with large nuclei having multiple nucleoli. Cytoplasmic blebbing seen is due to cytospin artifact (May Grunwald Giemsa, 400x; Inset: Papanicolaou, 400x)

Discussion

Hypopyon may develop in acute lymphoblastic leukemia,1234 acute myeloid leukemia and chronic myeloid leukemia. In ALL, anterior segment involvement has been estimated at 2.5 to 18% of relapsed cases, depending on the stage of the disease.2 The mechanisms by which the cells migrate into the anterior chamber are not clear. One hypothesis holds that the cells are sequestered in the long posterior ciliary vessels and pass into the anterior chamber through the iris vessels.1 Central nervous system (CNS) is the one of the most frequent sites of relapse after initial induction of remission. Prophylactic therapy to the CNS and posterior pole of the eye has usually been advocated as the blood brain barrier restricts free passage of certain chemotherapeutic agents and the eye, like the CNS, is a pharmacologic sanctuary.156 Before the advent of prophylactic treatment of childhood leukemia by means of CNS irradiation and intrathecal methotrexate, CNS leukemic relapse rate was high.7

A hypopyon in a child would make us suspicious of a masquerade syndrome. Prompt anterior chamber paracentesis and pathological studies should be done in such cases where history of leukemia is present even though other systemic investigations might indicate remission. Complete ocular examination including slit-lamp examination should be performed in all leukemic patients periodically. Early diagnosis is the aim to detect such extramedullary relapses so that timely referral and effective treatment can be initiated.

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2. Yi DH, Rashid S, Cibas ES, Arrigg PG, Dana MR. Acute unilateral leukemic hypopyon in an adult with relapsing acute lymphoblastic leukemia Am J Ophthalmol. 2005;139:719–21
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7. Evans AE, Gilbert ES, Zandstra R. The increasing incidence of central nervous system leukemia in children.(Children's Cancer Study Group A) Cancer. 1970;26:404–9
Keywords:

Acute lymphoblastic leukemia; hypopyon; relapse

© 2007 Indian Journal of Ophthalmology | Published by Wolters Kluwer – Medknow