Ocular granulocytic sarcoma (chloroma) with acute myelomonocytic leukemia in Turkish children

Abstract

In a series of 166 leukemic children from Turkey, 56 had acute myelomonocytic leukemia (AMML). Seventeen boys and 3 girls presented with chloroma-like deposits (granulocytic or myeloid sarcomas) in the eye and orbit, all showing AMML on initial study of blood and marrow. The ocular lesions responded rapidly to antileukemic therapy. Laboratory studies of AMML cases revealed no cytogenetic or immune defects, and Epstein-Barr virus titers were normal. A group-specific (GS-3) antigen (type-C virus?) was identified in one patient by radioimmunoassay of orbital tumor extracts. It is not clear what factors contribute toward the myelomoncytic differentiation of leukemia and its localization in the eye and orbit, but opportunities for further study are enhanced by reports of a predisposition to ocular chloroma among leukemic children in Africa, Egypt, and Japan.