title: Increased glycosylphosphatidylinositol-anchored protein-deficient granulocytes define a benign subset of bone marrow failures in patients with trisomy 8.
abstract: Trisomy 8 (+8), one of the most common chromosomal abnormalities found in patients with myelodysplastic syndromes (MDS), is occasionally seen in patients with otherwise typical aplastic anemia (AA). Although some studies have indicated that the presence of +8 is associated with the immune pathophysiology of bone marrow (BM) failure, its pathophysiology may be heterogeneous. We studied 53 patients (22 with AA and 31 with low-risk MDS) with +8 for the presence of increased glycosylphosphatidylinositol-anchored protein-deficient (GPI-AP(-) ) cells, their response to immunosuppressive therapy (IST), and their prognosis. A significant increase in the percentage of GPI-AP(-) cells was found in 14 (26%) of the 53 patients. Of the 26 patients who received IST, including nine with increased GPI-AP(-) cells and 17 without increased GPI-AP(-) cells, 14 (88% with increased GPI-AP(-) cells and 41% without increased GPI-AP(-) cells) improved. The overall and event-free survival rates of the +8 patients with and without increased GPI-AP(-) cells at 5 yr were 100% and 100% and 59% and 57%, respectively. Examining the peripheral blood for the presence of increased GPI-AP(-) cells may thus be helpful for choosing the optimal treatment for +8 patients with AA or low-risk MDS.
authors.affiliation: Cellular Transplantation Biology, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan.
authors.forename: Kohei
authors.initials: K
authors.lastname: Hosokawa
authors.affiliation: Cellular Transplantation Biology, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan.
authors.forename: Naomi
authors.initials: N
authors.lastname: Sugimori
authors.affiliation: Clinical Laboratory Science, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan.
authors.forename: Takamasa
authors.initials: T
authors.lastname: Katagiri
authors.affiliation: Cellular Transplantation Biology, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan.
authors.forename: Yumi
authors.initials: Y
authors.lastname: Sasaki
authors.affiliation: Cellular Transplantation Biology, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan.
authors.forename: Chizuru
authors.initials: C
authors.lastname: Saito
authors.affiliation: Cellular Transplantation Biology, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan.
authors.forename: Yu
authors.initials: Y
authors.lastname: Seiki
authors.affiliation: Cellular Transplantation Biology, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan.
authors.forename: Kanako
authors.initials: K
authors.lastname: Mochizuki
authors.affiliation: Cellular Transplantation Biology, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan.
authors.forename: Hirohito
authors.initials: H
authors.lastname: Yamazaki
authors.affiliation: Cellular Transplantation Biology, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan.
authors.forename: Akiyoshi
authors.initials: A
authors.lastname: Takami
authors.affiliation: Cellular Transplantation Biology, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan.
authors.forename: Shinji
authors.initials: S
authors.lastname: Nakao
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000741:Anemia, Aplastic; D001853:Bone Marrow / Q000378:metabolism / Q000473:pathology; D001855:Bone Marrow Diseases; D000080983:Bone Marrow Failure Disorders; D002898:Chromosomes, Human, Pair 8; D005260:Female; D058851:GPI-Linked Proteins / Q000172:deficiency* / Q000378:metabolism; D006098:Granulocytes / Q000378:metabolism*; D006457:Hemoglobinuria, Paroxysmal / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000235:genetics* / Q000378:metabolism* / Q000401:mortality; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015994:Incidence; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome; D014314:Trisomy; D055815:Young Adult
chemical_list: D058851:GPI-Linked Proteins; D007166:Immunosuppressive Agents
keywords: GPI-AP− cells; bone marrow failure; immunosuppressive therapy; trisomy 8