Herein is described a case of retinochoroidal infarction during re-induction chemotherapy for biphenotypic acute lymphoblastic leukemia (ALL). Retinochoroidal infarction is uncommon in ALL patients. However, some serious problems of this complication, which pediatric oncologists should be aware of, are illustrated in the present case.
Case reportA 4-year-old girl was admitted to Daisan Hospital, Jikei University School of Medicine, Tokyo because of fever, purpura and pale facial appearance. Her blood examination results showed anemia and thrombocytopenia. Her white blood cell count was elevated to 23 500/ L, containing 70% blasts in peripheral blood. In her bone marrow, mononuclear cells totalled 123 000 L and atypical lymphoblasts dominated at 96%. She was diagnosed with ALL, L1, based on the French -US -British classifi cation. Immunologically, CD 10, 19, 13, 33 and human leukocyte antigen-DR were positive in her leukemic blasts, which indicates biphenotypic ALL. Induction chemotherapy was started based on the Tokyo Children's Cancer Study Group (TCCSG) protocol for high-risk patients of ALL. 1 One week later her treatment was changed to an extremely highrisk regimen because of resistance to the initial steroid treatment. Complete remission (CR) was achieved on day 49 and all scheduled chemotherapy was completed in 14 months. Five months after the completion of chemotherapy, she relapsed. Re-induction chemotherapy in combination with l-asparaginase (l-asp), vincristine, daunorubicin and prednisolone was started based on the TCCSG protocol for the relapsed ALL. 1 During re-induction chemotherapy, three incidents of sepsis; pre-shock caused by l-asparaginase; pancreatitis; transient glucosuria; low level of antithrombin III (ATIII; minimum: 47%); and fi brinogen (minimum: 56 mg/dL); psychological symptoms; hypertension; stomatitis ; and liver dysfunction occurred. She was compensated with platelet (total: 105 units), red blood cell transfusion (total: 10 units) and ATIII concentrates (total: 3500 units). All of these symptoms were transient and improved. The second CR was achieved on day 62. On day 63, an exotropia in her right eye was noticed by her family. Her decimal visual acuity was 0.01 in the right and 1.2 in the left. Retinal edema, soft exudates and a degeneration on the right macular spot and old chorioretinal atrophy on the left were revealed by an ophthalmologic fundus examination. No infi ltration of leukemic cells, infection or hemorrhage was identifi ed ( Fig. 1). Interruption of blood fl ow on the macular spot was shown with fl uorescein angiography in the early stage. And hyperfl uorescence was shown in the same area and enhanced continuously during the late stage ( Fig. 2). The fi nal diagnosis was retinochoroidal infarction. Her brain magnetic resonance imaging, cerebroid fl uid and hematological data were not identifi ed with the tumor involvement. Her physical, ophthalmologic and other laboratory fi ndings were not identifi ed with any infectious pathogen. This ophthalmologic complication was...