title: T-cell large granular lymphocytic leukemia and hereditary hemochromatosis: a fortuitous association?
abstract: We describe a patient with hereditary hemochromatosis (homozygous for C282Y mutation) and neutropenia who was found to have underlying T-cell large granular lymphocytic (T-LGL) leukemia. The diagnosis was confirmed by demonstrating T-cell receptor (TCR) gene rearrangement by polymerase chain reaction (PCR). Multiple quantitative and qualitative defects have been described for the T cells of patients with hemochromatosis. Although the association between the two may be fortuitous, this case report raises the possibility that the T cells in these patients may be susceptible to leukemic transformation as well.
authors.affiliation: Department of Medicine, Caritas St. Elizabeth's Medical Center, Tufts University School of Medicine, Boston, Massachusetts 07102, USA. sumitagaurmd@hotmail.com
authors.forename: S
authors.initials: S
authors.lastname: Gaur
authors.forename: S
authors.initials: S
authors.lastname: Mansoor
authors.forename: L
authors.initials: L
authors.lastname: Aish
mesh_terms: D005260:Female; D005293:Ferritins / Q000097:blood; D015329:Gene Rearrangement, T-Lymphocyte; D006432:Hemochromatosis / Q000097:blood / Q000150:complications* / Q000235:genetics*; D006801:Humans; D015458:Leukemia, T-Cell / Q000097:blood / Q000150:complications*; D008875:Middle Aged; D018962:Phlebotomy; D016896:Treatment Outcome
chemical_list: D005293:Ferritins