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Sickle cell crisis associated with hemophagocytic lymphohistiocytosis
Author(s) -
Kio Ebenezer,
Onitilo Adedayo,
Lazarchick John,
Hanna Maged,
Brunson Chris,
Chaudhary Uzair
Publication year - 2004
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.20198
Subject(s) - hepatosplenomegaly , hemophagocytic lymphohistiocytosis , medicine , hemoglobinopathy , sickle cell anemia , bone pain , thalassemia , bone marrow , beta thalassemia , ferritin , hemolytic anemia , disease , pediatrics , immunology
Sickle‐beta + (β + ) 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. Am. J. Hematol. 77:229–232, 2004. © 2004 Wiley‐Liss, Inc.