To the editor: A 25-year-old male had been well until October 2009, when he developed abdominal pain, diarrhea, and fever. The results of the hematological examination were as follows. White blood cells were 108.76×109/L. The bone marrow was hypercellular with 32.5% lymophoblasts and 55.5% infantile lymphocytes which were cytochemically positive for PAS. Flow cytometric analysis revealed that the blasts were positive for CD7, CD2, CD3, CD8, TCR-α-β-1, CD38, CD19, CD13, pgP, CD56, cyCD79a, and CD117. Cytogenetic examination showed 45,XY,-8,der(9)t(8;9)(q12,p12) /46,XY. Finally, a diagnosis of biphenotypic acute leukemia (T/B) was made. Meanwhile, the patient suffered from disseminated intravascular coagulation, and recovered after treatment of fibrinogen. Then, he started receiving chemotherapy and got hematologic complete remission after the second session. Nevertheless, the central nervous system (CNS) leukemia was discovered. Immediately, he was treated successively with intrathecal injections for six times. The flow cytometry of bone marrow and cerebrospinal fluid were negative after the fifth course of chemotherapy. A cycle of consolidation treatment was followed. Later, the patient prepared to undergo an allogeneic stem cell transplant. Though, the next day after entering into the clean room, he presented with conjunctival congestion, blurred vision, and pain in the right eye. Morrow hypopyon had been identified. He was diagnosed as uveitis and secondary glaucoma. Fundus fluorescein angiography demonstrated iris neovascular of the right eye. Anterior chamber paracentesis was ultimately done in June 2010. On the third microscopic examination, a smear of the aspirated fluid showed a large number of blast cells (Figure 1). The flow cytometry showed that the proportion of abnormal cells to nucleated cells was 96.58%; the double mark masculine cell account for 64%, namely CD7 and CD19 expressions. Thus, leukemic infiltration of the anterior chamber had been confirmed. Presently, infiltration of the CNS and testicles also occurred. Then, the patient received multiple systemic chemotherapy and intrathecal chemotherapy. His ocular symptom was of a varying degree alleviating temporarily. About 5 months later, the patient experienced progressive lower extremity weakness and paresthesia. Magnetic resonance imaging revealed the optic nerves and thoracic spinal cord had also been infiltrated. Therefore, he underwent several doses of craniospinal axis irradiation and ocular irradiation. Despite these treatments, he still did not achieve prolonged remission and died in March 2011.
Ocular involvement in leukemia is unusual in patients with acute lymphoblastic leukemia (ALL).1,2 Anterior segment involvement has been estimated at 2.5 to 18% of relapsed ALL cases.3 The retina and choroid are the most common sites of ocular infiltration; the iris is rarely effected by leukemia.4 Systemic manifestations usually precede ocular manifestations.5 But hypopyon iridocylitis can also be a first and sole presentation of relapse. Most of the ophthalmic manifestations are related to bone marrow relapse and extramedullary infiltration, especially with CNS leukemia. Aspiration of fluid from the anterior chamber gives a rapid and definitive diagnosis.5 We advocate that paracentesis should be performed without delay in the anterior chamber when uveitis develops in leukemia, regardless of systemic relapse. Cytopathologic examination provides a definite diagnosis, the flow cytometry is a sensitive detection methods. The optimal treatment remains unknown and the prognosis is poor after leukemia infiltrates the anterior chamber.
1. Ramsay A, Lightman S. Hypopyon uveitis. Surv Ophthalmol 2001; 46: 1-18.
2. Wetzler M, Lincoff N. Unusual manifestations of acute leukemia. Case 3. Unilateral leukemia hypopyon. J Clin Oncol 2000; 18: 3439-3440.
3. 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: 719-721.
4. Robb RM, Ervin LD, Sallan SE. An autopsy study of eye involvement in acute leukemia of childhood. Med Pediatr Oncol 1979; 6: 171-177.
5. Dadeya S, Malik KP, Guliani BP, Dewan S, Mehta R, Gupta VS. Acute lymphocytic leukemia presenting as masquerade syndrome. Ophthalmic Surg Lasers 2002; 33: 163-165.