title: Low concentration of serum haptoglobin has impact on understanding complex pathophysiology in patients with acquired bone marrow failure syndromes.
abstract: To clarify whether measurement of serum haptoglobin (Hp) has impact on understanding pathophysiology in bone marrow failure (BMF) syndromes, we investigated concentrations of serum Hp by nephelometric procedure in 156 Japanese patients with BMF, including 54 aplastic anemia (AA), 50 paroxysmal nocturnal hemoglobinuria (PNH), and 52 myelodysplastic syndromes (MDS) patients. The frequencies with low concentrations of serum Hp (<42 mg/dL) in PNH patients (98.0%) were significantly higher than those in AA (27.8%; P < 0.0001) and MDS (38.5%; P < 0.0001) patients. In AA patients, white blood cell (WBC), absolute neutrophil, and platelet counts were significantly decreased in the group (n = 15) with low concentrations of serum Hp than in that (n = 39) with normal concentrations of it, and WBC counts were positively correlated with concentrations of serum Hp, suggesting that WBC counts may affect the concentrations. In MDS patients, hemoglobin concentrations and serum iron were significantly decreased and increased, respectively, in the group (n = 20) with low concentrations of serum Hp than in that (n = 32) with normal concentrations of it, and the values of serum iron were inversely correlated with concentrations of serum Hp, suggesting that ineffective erythropoiesis may affect the concentrations. Several AA and MDS patients with low concentrations of serum Hp had Coombs-negative autoimmune hemolytic anemia determined by immunoradiometric assay. In conclusion, several factors in conjunction with pathophysiology contribute to decrease of serum Hp in BMF.
authors.affiliation: Department of Cardiology and Hematology, Fukushima Medical University, Fukushima, Japan. t-shichi@fmu.ac.jp
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Shichishima
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Ikeda
authors.forename: Naoto
authors.initials: N
authors.lastname: Takahashi
authors.forename: Junichi
authors.initials: J
authors.lastname: Kameoka
authors.forename: Katsushi
authors.initials: K
authors.lastname: Tajima
authors.forename: Kazunori
authors.initials: K
authors.lastname: Murai
authors.forename: Yoshiko
authors.initials: Y
authors.lastname: Tamai
authors.forename: Akiko
authors.initials: A
authors.lastname: Shichishima-Nakamura
authors.forename: Kazuko
authors.initials: K
authors.lastname: Akutsu
authors.forename: Hideyoshi
authors.initials: H
authors.lastname: Noji
authors.forename: Masatoshi
authors.initials: M
authors.lastname: Okamoto
authors.forename: Hideo
authors.initials: H
authors.lastname: Kimura
authors.forename: Hideo
authors.initials: H
authors.lastname: Harigae
authors.forename: Takashi
authors.initials: T
authors.lastname: Oyamada
authors.forename: Toyomi
authors.initials: T
authors.lastname: Kamesaki
authors.forename: Yasuchika
authors.initials: Y
authors.lastname: Takeishi
authors.forename: Kenichi
authors.initials: K
authors.lastname: Sawada
mesh_terms: D000328:Adult; D000368:Aged; D000741:Anemia, Aplastic / Q000097:blood / Q000235:genetics / Q000503:physiopathology; D000744:Anemia, Hemolytic, Autoimmune / Q000097:blood / Q000235:genetics / Q000503:physiopathology; D001855:Bone Marrow Diseases / Q000097:blood* / Q000235:genetics / Q000503:physiopathology*; D003165:Complement System Proteins / Q000378:metabolism; D003298:Coombs Test; D004912:Erythrocytes / Q000502:physiology; D005260:Female; D005787:Gene Frequency; D006239:Haplotypes; D006242:Haptoglobins / Q000235:genetics / Q000378:metabolism*; D006410:Hematopoiesis / Q000502:physiology; D006457:Hemoglobinuria, Paroxysmal / Q000097:blood / Q000235:genetics / Q000503:physiopathology; D006461:Hemolysis / Q000502:physiology; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D008297:Male; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000097:blood / Q000235:genetics / Q000503:physiopathology
chemical_list: D006242:Haptoglobins; D007074:Immunoglobulin G; D003165:Complement System Proteins