Large granular lymphocyte leukemia (LGL) in a child with hyper IgM syndrome and autoimmune hemolytic anemia.
We describe a female with a history of autosomal recessive hyper-IgM (HIGM) syndrome along with a history of autoimmune hemolytic anemia and intermittent lymphadenopathy. She subsequently developed neutropenia, lymphocyostosis and mild thrombocytopenia. Flow cytometry of the peripheral blood revealed the presence of a marked predominance of cytotoxic T lymphocytes, shown to be clonal, with concomitant natural killer (NK) antigen expression. She responded to weekly methotrexate therapy.
Kitchen
Brenda J
BJ
Division of Hematology/Oncology, Department of Pediatrics and Communicable Diseases, University of Michigan, Mott Children's Hospital, Ann Arbor, Michigan, USA. nkbrenda@umich.edu
Boxer
Laurence A
LA
D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications*; D002648:Child; D005260:Female; D006801:Humans; D053306:Hyper-IgM Immunodeficiency Syndrome / Q000150:complications*; D054066:Leukemia, Large Granular Lymphocytic / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy