Hypoplastic Myelodysplastic Syndrome (h-MDS) Is a Distinctive Clinical Entity with Poorer Prognosis and Frequent Karyotypic and FISH Abnormalities Compared to Aplastic Anemia (AA).

Abstract

Abstract 3199Poster Board III-136Differences between hypoplastic MDS (h-MDS) and aplastic anemia (AA) are not defined. Role of karyotype and fluorescent in situ hybridization (FISH) in these diseases is not established.Medical record review at Seoul National University Hospital between 1990 and 2008 was performed. Patients diagnosed as either h-MDS or AA based on morphology was reviewed. We assessed overall survival (OS) and leukemic conversion.369 AA and 40 h-MDS patients (median age 39 years, range 15-82) were analyzed. 235 and 165 patients underwent karyotyping and FISH at diagnosis respectively. Compared to AA, karyotypic abnormality, 5q deletion, trisomy 8 and trisomy 1q FISH abnormalities were more frequently found in h-MDS. Median OS of h-MDS was shorter than AA (60 vs. 219 months, p<0.001) with prognosis of h-MDS falling between severe and very severe AA. Patients with h-MDS had more frequent leukemic conversion (p<0.001) than AA patients. Karyotypic abnormality was not prognostic in AA (p=0.225). For h-MDS, complex karyotype and trisomy 1q FISH abnormality predicted poor prognosis.The prognosis of h-MDS falls between severe and very severe AA. h-MDS accompanies frequent karyotypic and FISH abnormality and is prone to leukemic conversion. Complex karyotype and trisomy 1q FISH abnormality may have a prognostic role in h-MDS.Table 1Characteristics of patients according morphologic classification in 409 patientsCharacteristicsAplastic anemia (N=369)Hypoplastic MDS (N=40)p-valueAge (median)38.049.50.005Gender0.846Male19722Female17218White blood cell (mean) (/μL)272430420.368Reticulocyte count (mean) (%)1.11.80.007Hemoglobin (mean) (g/dL)8.37.90.357Platelet (mean) (/μL)441301090000.008Severity of cytopenia*0.007Moderate24436Severe964Very severe280PNH componentNAPresent15NAAbsent351NAIPSS risk categoryNAIntermediate-1NA25Intermediate-2NA4HighNA1Oxymetholone administration#0.005Yes1679No19630Immunosuppressive treatment#0.005Yes1144No24935Stem cell transplantation#1.000Yes495No31434 [Display omitted] DisclosuresNo relevant conflicts of interest to declare.