title: Diagnosis of acquired bone marrow failure syndrome during childhood using the 2008 World Health Organization classification system.
abstract: Distinguishing hypoplastic myelodysplastic syndrome from aplastic anemia (AA) is challenging. In the present study, Japanese and Chinese pediatric hematologists and pathologists conducted a joint review of bone marrow (BM) smears and trephine biopsies in 100 children with acquired BM failure syndrome, using the criteria proposed in the 2008 edition of the World Health Organization classification of hematopoietic and lymphoid tissues. The final consensus for the diagnoses of 100 children was AA in 29 patients, refractory cytopenia of childhood (RCC) in 58 patients, and refractory cytopenia with multilineage dysplasia (RCMD) in 13 patients. No significant differences between Japanese and Chinese children were found with regards to clinical and laboratory findings, or the distribution of diagnoses. Patients with RCC/RCMD showed milder disease severity and BM hypocellularity than those with AA. To establish the provisional entities for RCC, it is essential to prospectively compare the clinical outcomes between AA and RCC groups in a large number of patients.
authors.affiliation: Diagnostic and Therapeutic Center of Children's Blood Disease, Blood Disease Hospital, Institute of Hematology, Chinese Academy of Medical Sciences, 288 Nanjing Road, Tianjin, China.
authors.forename: Wenyu
authors.initials: W
authors.lastname: Yang
authors.forename: Peihong
authors.initials: P
authors.lastname: Zhang
authors.forename: Asahito
authors.initials: A
authors.lastname: Hama
authors.forename: Masafumi
authors.initials: M
authors.lastname: Ito
authors.forename: Seiji
authors.initials: S
authors.lastname: Kojima
authors.forename: Xiaofan
authors.initials: X
authors.lastname: Zhu
mesh_terms: D000293:Adolescent; D000741:Anemia, Aplastic / Q000145:classification / Q000175:diagnosis*; D000753:Anemia, Refractory / Q000145:classification / Q000175:diagnosis*; D044466:Asian People; D001855:Bone Marrow Diseases; D000080983:Bone Marrow Failure Disorders; D002648:Child; D002675:Child, Preschool; D002681:China; D003937:Diagnosis, Differential; D005260:Female; D006457:Hemoglobinuria, Paroxysmal / Q000145:classification / Q000175:diagnosis*; D006801:Humans; D007223:Infant; D007564:Japan; D008297:Male; D014944:World Health Organization