A rare case report of hemophagocytic lymphohistiocytosis secondary to pure eythroid leukemia in a person living with HIV

Pure erythroid leukemia (AML-M6) is a rare variant of acute myeloid leukemia (AML) with predominant erythroid lineage proliferation. The incidence of AIDS defining cancers including Kaposi sarcoma and non-Hodgkins lymphoma are on declining trends due to effective use of HAART (Highly Active Antiretroviral Therapy). Correspondingly, there have been increasing cases of leukemia in persons living with HIV. Our case is a 43 years old male living with HIV who was admitted with high grade fever and mucosal bleeds. On examination, he had periorbital swelling and ecchymosis with hepatosplenomegaly. The laboratory investigations revealed bicytopenia with high ferritin, low fibrinogen and hypertriglyceridemia. A diagnosis of hemophagocytic lymphohistiocytosis (HLH) with H score of 222 was made. Bone marrow examination revealed hypercellular marrow with more than 80% cells of erythroid lineage with 47% proerythroblasts. Suggesting pure erythroid leukemia (AML-M6). This diagnosis with secondary HLH carries a very poor prognosis in persons living with HIV.