ABSTRACT
<p><b>BACKGROUND</b>Patients with myelodysplastic syndrome (MDS) display a very diverse pattern. In this study, we investigated prognostic factors and survival rate in adult patients with MDS refractory anaemia (MDS-RA) diagnosed according to French-American-British classification and evaluated the International Prognostic Scoring System (IPSS) for Chinese patients.</p><p><b>METHODS</b>A multi-center study on diagnosis of MDS-RA was conducted to characterize the clinical features of Chinese MDS patients. The morphological criteria for the diagnosis of MDS-RA were first standardized. Clinical data of 307 MDS-RA patients collected from Shanghai, Suzhou and Beijing from 1995 to 2006 were analyzed using Kaplan-Meier curve, log rank and Cox regression model.</p><p><b>RESULTS</b>The median age of 307 MDS-RA cases was 52 years. The frequency of 2 or 3 lineage cytopenias was 85.6%. Abnormal karyotype occurred in 35.7% of 235 patients. There were 165 cases (70.2%) in the good IPSS cytogenetic subgroup, 44 cases (18.7%) intermediate and 26 cases (11.1%) poor. IPSS showed 20 (8.5%) categorized as low risk, 195 cases (83.0%) as intermediate-I risk and 20 cases (8.5%) as intermediate-II risk. The 1-, 2-, 3-, 4- and 5-year survival rates were 90.8%, 85.7%, 82.9%, 74.9% and 71.2% respectively. Fifteen cases (4.9%) transformed to acute myeloid leukaemia (median time 15.9 months, range 3 - 102 months). Lower white blood cell count (< 1.5 x 10(9)/L), platelet count (< 30 x 10(9)/L) and cytogenetic abnormalities were independent prognostic factors by multivariate analysis, but age (= 65 years), IPSS cytogenetic subgroup and IPSS risk subgroup were not independent prognostic factors associated with survival time.</p><p><b>CONCLUSIONS</b>Chinese patients were younger, and had lower incidence of cytogenetic abnormalities, more severe cytopenias but a more favourable prognosis than Western patients. The major prognostic factors were lower white blood cell count, lower platelet count and fewer abnormal karyotypes. The international prognostic scoring system risk group was not an independent prognostic factor for Chinese myelodysplastic syndrome patients with refractory anaemia patients.</p>
Subject(s)
Adolescent , Adult , Aged , Aged, 80 and over , Child , Female , Humans , Male , Middle Aged , Anemia, Refractory , Mortality , Asian People , China , Myelodysplastic Syndromes , Mortality , PrognosisABSTRACT
In order to study whether erythroleukemia was really a subtype of acute leukemia, the clinical laboratory characteristics and development of disease in 21 cases of erythroleukemia were analyzed. The results indicated that the percentage of patients with leucocytopenia, anemia and thrombocytopenia were 42.9%, 81% and 81% respectively at the time of diagnosis. These were 85.7% of patients with myelocytes and premonocyte, 52.4% of patients with erythroblast in their blood smear respectively. All of the bone marrow showed active or significantly active proliferation. The median percentage of erythro-lineage, myeloblast of NEC and displasia were (58.3 +/- 8.0)%, (58.0 +/- 18.4)% and 66.7% respectively, that is different from typical AML. 52.4% of M(6) patients transferred to RAEB/RAEB-T and AML-M(2) subtype in the disease progression. 11/19 cases (57.4%) achieved remission (CR 10; PR 1) after chemotherapy. The median remission length were 6 months for CR patients and 2 months for PR patients, but most of CR patients displayed obvious displasia of bone marrow and cytopenia of blood in the period of CR. The median survival length of M(6) and MDS-->M(6) from time of diagnosis were 13.0 +/- 13.2 and 2.3 +/- 1.3 months respectively. It is concluded that there are differences between M(6) and typical AML. Most of M(6) patients would rather be classified MDS RAEB and RAEB-t with over-hyperplasia of erythron lineage than a subtype of AML.