Acute Pure Erythroid Leukemia Presenting with Chloroma in a Child: A Case Report with Review of Literature

Abstract Background: Pure erythroid leukemia (PEL) is the rarest subtype of acute myeloid leukemia (AML), characterized by clonal proliferation of cells of erythroid origin with minimal myeloblast proliferation. It is typically a disease of the adults and presentation in children in association with chloroma is extremely rare. Clinical Description: A 20-month-old boy presented with left eye proptosis, and fever for a month, with bleeding from the eye for 10 days. He was febrile with pallor, and a nontender, nonfluctuant swelling with subconjunctival hemorrhage in the left eye and hepatomegaly. Management and Outcome: Investigations showed anemia and thrombocytopenia with 30% blasts in peripheral smear. Bone marrow examination was suggestive of acute leukemia and immunophenotype was confirmatory for PEL. The child was treated with cytarabine, doxorubicin, and etoposide during induction phase followed by high-dose cytarabine consolidation. The child remained in remission for 6 months, after which his disease relapsed in bone marrow and the family opted for palliative care. Conclusion: Rapidly developing nontender unilateral proptosis with bleeding may be a manifestation of chloroma associated with PEL. A combination of bone marrow, immunophenotyping, and karyotyping is required for its diagnosis. Conventional therapy for AML is poor.