title: Oral cyclophosphamide was effective for Coombs-negative autoimmune hemolytic anemia in CD16+CD56- chronic lymphoproliferative disorder of NK-cells.
abstract: An 84-year-old woman was referred to our hospital presenting anemia. Her hemoglobin level was 5.8 g/dL, and white blood cell count was 9400/μL, consisting of 82% lymphocytes. Given the lymphocyte phenotype (CD2+, CD3-, CD16+, and CD56-) and negative whole blood EBV viral load, we made a diagnosis of chronic lymphoproliferative disorder of NK cells (CLPD-NK). We suspected hemolytic anemia because of the high levels of reticulocytes in the peripheral blood and the low haptoglobin value. Although the direct Coombs test was negative and there was no cold agglutination, we examined her red-blood-cell-bound IgG (RBC-IgG), which was elevated. She was diagnosed as having as Coombs-negative autoimmune hemolytic anemia (AIHA). We report the effectiveness of oral cyclophosphamide for Coombs-negative autoimmune hemolytic anemia in CLPD-NK.
authors.affiliation: Department of Comprehensive Cancer Therapy, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano, 390-8621, Japan. nodokas@shinshu-u.ac.jp.|Division of Hematology, Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Nagano, Japan. nodokas@shinshu-u.ac.jp.
authors.forename: Nodoka
authors.identifier: http://orcid.org/0000-0002-0384-9611
authors.initials: N
authors.lastname: Sekiguchi
authors.affiliation: Division of Hematology, Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Nagano, Japan.
authors.forename: Sayaka
authors.initials: S
authors.lastname: Nishina
authors.affiliation: Division of Hematology, Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Nagano, Japan.
authors.forename: Toru
authors.initials: T
authors.lastname: Kawakami
authors.affiliation: Division of Hematology, Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Nagano, Japan.
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Sakai
authors.affiliation: Division of Hematology, Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Nagano, Japan.
authors.forename: Noriko
authors.initials: N
authors.lastname: Senoo
authors.affiliation: Division of Hematology, Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Nagano, Japan.
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Senoo
authors.affiliation: Division of Hematology, Matsumoto Medical Center Masumoto Hospital, Matsumoto, Nagano, Japan.
authors.forename: Toshiro
authors.initials: T
authors.lastname: Ito
authors.affiliation: Department of Internal Medicine, Nagano Prefectural Suzaka Hospital, Suzaka, Nagano, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Saito
authors.affiliation: Division of Hematology, Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Nagano, Japan.
authors.forename: Hideyuki
authors.initials: H
authors.lastname: Nakazawa
authors.affiliation: Department of Comprehensive Cancer Therapy, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano, 390-8621, Japan.
authors.forename: Tomonobu
authors.initials: T
authors.lastname: Koizumi
authors.affiliation: Division of Hematology, Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Nagano, Japan.|Department of Biomedical Laboratory Medicine, Shinshu University School of Medicine, Matsumoto, Nagano, Japan.
authors.forename: Fumihiro
authors.initials: F
authors.lastname: Ishida
mesh_terms: D000369:Aged, 80 and over; D000744:Anemia, Hemolytic, Autoimmune / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy; D001323:Autoantibodies / Q000097:blood; D019002:CD56 Antigen / Q000097:blood; D002908:Chronic Disease; D003520:Cyclophosphamide / Q000008:administration & dosage*; D005260:Female; D058851:GPI-Linked Proteins / Q000097:blood; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D007694:Killer Cells, Natural / Q000378:metabolism*; D008232:Lymphoproliferative Disorders / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy; D017452:Receptors, IgG / Q000097:blood; D012156:Reticulocytes / Q000378:metabolism
chemical_list: D001323:Autoantibodies; D019002:CD56 Antigen; C428200:FCGR3B protein, human; D058851:GPI-Linked Proteins; D007074:Immunoglobulin G; C522918:NCAM1 protein, human; D017452:Receptors, IgG; D003520:Cyclophosphamide
keywords: Chronic lymphoproliferative disorder of NK cells; Coombs-negative autoimmune hemolytic anemia; Pure red cell aplasia; Signal transducer and activator of transcription 3