title: Acute myelocytic leukemia in a patient with hemophagocytic lymphohistiocytosis: A case report.
abstract: Hemophagocytic lymphohistiocytosis (HLH), also known as hemophagocytic syndrome, is an aggressive hyperinflammatory condition characterized by prolonged fever, cytopenias and hepatosplenomegaly, as well as hemophagocytosis by activated, morphologically benign macrophages. HLH may be characterized into two forms, familial and secondary HLH. Familial HLH usually manifests in children with genetic abnormalities associated with the cytotoxic function of NK and T cells, whereas secondary HLH usually occurs in older patients in combination with an associated condition, such as infection or malignancy, without an identifiable genetic abnormality. Malignancy-associated hemophagocytic lymphohistiocytosis is mostly accompanied by lymphoid neoplasms. The present study reports a rare case of this syndrome in combination with acute myeloblastic leukemia (AML-M2), in a patient with clonal karyotypic abnormalities. The patient was successfully treated with chemotherapy comprising daunorubicin (40 mg/m2i.v., days 1-3) and cytosine arabinoside (100 mg/m2, 1-h i.v. infusion, days 1-7). All clinical symptoms disappeared following chemotherapy.
authors.affiliation: Department of Hematology, Navy General Hospital, Beijing 100048, P.R. China.
authors.forename: Defeng
authors.initials: D
authors.lastname: Zhao
authors.affiliation: Department of Hematology, Navy General Hospital, Beijing 100048, P.R. China.
authors.forename: Liren
authors.initials: L
authors.lastname: Qian
authors.affiliation: Department of Hematology, Navy General Hospital, Beijing 100048, P.R. China.
authors.forename: Jianliang
authors.initials: J
authors.lastname: Shen
keywords: chemotherapy; hemophagocytic lymphohistiocytosis; leukemia