title: Granular lymphocyte leukemia with pure red cell aplasia: usefulness of gene analysis in assessing therapeutic effect.
abstract: A patient with granular lymphocyte leukemia (GLL) of the CD3+, CD4-, CD8+ phenotype accompanied by pure red cell aplasia (PRCA) is described. Surface marker analysis, nonmajor histocompatibility complex (MHC)-restricted cytotoxicity assay, gene analysis, and in vitro colony assay were performed on the granular lymphocytes before and after treatment. Cyclophosphamide therapy was highly effective, and after remission clonal granular lymphocytes were no longer identified by T-cell antigen receptor (TCR) gene analysis or surface marker analysis. Lymphocytes obtained after remission did not exhibit elevated levels of non-MHC-restricted cytotoxicity, nor did they demonstrate a suppressive effect on erythroid colony formation. TCR gene analysis proved to be a sensitive parameter for evaluating the residual malignant granular lymphocytes. Gene analysis will be useful both for timing the discontinuation of treatment and for the early detection of relapse. Various factors possibly related to the development of PRCA in this patient were investigated and their significance is discussed.
authors.affiliation: Department of Medicine, Tokyo Women's Medical College, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Motoji
authors.forename: O
authors.initials: O
authors.lastname: Yamada
authors.forename: M
authors.initials: M
authors.lastname: Takahashi
authors.forename: K
authors.initials: K
authors.lastname: Oshimi
authors.forename: H
authors.initials: H
authors.lastname: Mizoguchi
mesh_terms: D001853:Bone Marrow / Q000473:pathology; D003470:Culture Media; D003520:Cyclophosphamide / Q000627:therapeutic use; D015672:Erythroid Precursor Cells / Q000187:drug effects; D005260:Female; D005544:Forecasting; D015333:Gene Rearrangement, beta-Chain T-Cell Antigen Receptor / Q000187:drug effects; D005821:Genetic Techniques; D006801:Humans; D007945:Leukemia, Lymphoid / Q000150:complications* / Q000188:drug therapy / Q000235:genetics; D007951:Leukemia, Myeloid / Q000150:complications* / Q000188:drug therapy / Q000235:genetics; D008875:Middle Aged; D009000:Monocytes / Q000502:physiology; D009364:Neoplasm Recurrence, Local; D012010:Red-Cell Aplasia, Pure / Q000150:complications* / Q000188:drug therapy
chemical_list: D003470:Culture Media; D003520:Cyclophosphamide