Título

ACUTE LYMPHOBLASTIC LEUKEMIA RELAPSE IN THE IRIS AND CILIARY BODY

Objetivo

The acute lymphoblastic leukemia (ALL) is the most common pediatric cancer. Despite treatments advances, it must relapse in 20% of patients. Leukemic infiltration of the anterior chamber (AC) is uncommon and is associated with poorly prognosis. We describe a rare case of iris and ciliary body infiltration in ALL.

Relato do Caso

A 11-years-old girl with B-cell progenitor ALL complained of one month blurred vision and left leukocoria. Symptoms initiated 17 months after diagnosis, at the maintenance scheme according to BFM 2009 chemotherapic regimen. On ocular examination, BCVA was 20/20 in the right eye and 20/25 in the left eye. Left eye presented a decreased pupillary light reflex, small keratic precipitates, 4+ AC reaction with pseudohypopyon, and iris thickening predominantly from 1- to 9-o’clock. Left eye fundoscopy and B-scan ultrasound were unremarkable. Ultrasound biomicroscopy showed thickness of the iris and ciliary body, associated with a cyst of the iris pigment epithelium at 6-o’clock position. Cerebrospinal fluid immunophenotyping revealed 71% of B-lymphoid blasts and aqueous humor cytology showed leukemic blasts, confirming the early relapse. The remission induction chemotherapy with II-IDA protocol was initiated, according to the ALL-REZ BFM 2002. The BCVA improved to 20/20 in both eyes and the pseudohypopyon, AC reaction and the iris and ciliary body thickness decreased. The patient awaits the possibility of the allogeneic hematopoietic stem cell transplantation.

Conclusão

The ALL is a relatively common cancer in children. However, iris and ciliary body infiltration are rare. The management of intermediate-risk group of relapsed ALL, as in the case reported, is a challenge. In some cases, intensification of chemotherapic regimen can regress the lesions but radiation therapy, despite its side effects, is current used for ocular ALL. We report a very rare case of iris and ciliary body infiltration as relapse sites of ALL.