title: Sickle cell crisis associated with hemophagocytic lymphohistiocytosis.
abstract: Sickle-beta(+) (beta(+)) thalassemia is a double heterozygous genetic disorder characterized by both a qualitative and quantitative abnormality. We present a case of an African American male who was first diagnosed with sickle cell disease (SCD) at the age 23 years when he presented with generalized bone pain, fever, and hepatosplenomegaly. Laboratory findings included thrombocytopenia, microcytic anemia, and markedly elevated ferritin. He was subsequently diagnosed with a sickle-beta thalassemia hemoglobinopathy. Findings in the bone marrow aspirate and biopsy were consistent with hemophagocytic lymphohistiocytosis (HLH). HLH resolved with the resolution of sickle cell bone pain crisis without use of immunosuppressive therapy. To the best of our knowledge this is the first documented case of HLH associated with sickle cell bone pain crisis.
authors.affiliation: Hematology/Oncology Division, Medical University of South Carolina, Charleston, South Carolina 2425, USA.
authors.forename: Ebenezer
authors.initials: E
authors.lastname: Kio
authors.forename: Adedayo
authors.initials: A
authors.lastname: Onitilo
authors.forename: John
authors.initials: J
authors.lastname: Lazarchick
authors.forename: Maged
authors.initials: M
authors.lastname: Hanna
authors.forename: Chris
authors.initials: C
authors.lastname: Brunson
authors.forename: Uzair
authors.initials: U
authors.lastname: Chaudhary
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000473:pathology; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D002443:Ceftriaxone / Q000627:therapeutic use; D015616:Histiocytosis, Non-Langerhans-Cell / Q000097:blood / Q000150:complications* / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male
chemical_list: D000900:Anti-Bacterial Agents; D002443:Ceftriaxone