A total of 30 myelodysplastic syndromes (MDS) patients was analyzed to determine the clinical aspects and erythroid morphology in peripheral blood and bone marrow. The results are summarized as follows: 63.3% of patients was (50 years old. Male/Female ratio: 0.8. 46.7% were with lonely anemia and the rest were anemia combined with hemorrhages, infections, hepatosplenomegaly and lymphadenopathy. In peripheral blood: 63.4% of patients was with Hb<60g/l. 50% had normocrom - normocytic erythrocyte 96.7% of patients had dysmorphologic erythrocyte. In bone marrow: 46.6% were with hyperplasia of erythroid lineage, 33.6% were with hypoplasia of erythroid lineage, the rest (20%) were with normoplasia of erythroid lineage, 63.4% had dysmorphologic erythroblast, in which 36.7% was with ringed syderoblast
Myelodysplastic Syndromes ; blood ; diagnosis ; cytology ; Erythrocytes ; anemia

OBJECTIVETo investigate whether serum ferritin (SF) level may be used as a indicator for predicting mortality of patients with myelodysplastic syndrome (MDS).

METHODSA total of 151 patients with MDS were followed up in our study, their blood routine indicators, bone marrow blasts and SF level were detected. All of the patients were divided into the dead group and survival group.

RESULTSThe average survival time of all patients was 30.0 ± 10.86 months. There were statistical differences in age, IPSS score, chromosome grouping and SF level between 2 groups (P < 0.05). COX model analysis showed that age, MDS type, IPSS score, chromosome grouping and SF level all were related with mortality of patients with MDS, which were risk factors of death for patients with MDS (P < 0.05). The receiver-operating characteristic curve (ROC) area for SF was 0.826 with a Cut off value of 622.95, the sensitivity and specificity was 77.5% and 75% respectively. Log-rank test showed that the mortalities of patients with different levels of SF were statistically and very significantly different (P < 0.01).

CONCLUSIONThe age, IPSS score, chromosome grouping and SF level closely correct with mortality of the patients with MDS, the SF level may be considered as a predictor of death for MDS patients.

Ferritins ; blood ; Humans ; Myelodysplastic Syndromes ; blood ; diagnosis ; Prognosis ; Risk Factors

Myelodysplastic syndromes (MDS) are a group of refractory anemias resulting from a clonal stem cell disorder often associated with cytogenetic abnormalities. There is increasing recognition of immunological abnormalities in patients with MDS, including defective B- and T-cell function, hyper- or hypogammaglobulinemia and monoclonal gammopathy. MDS have been associated with Sjogren's syndrome, polymyalgia rheumatica, relapsing polychondritis and systemic lupus erythematosus. Although there may be various rheumatologic features, including acute arthritis in MDS, chronic inflammatory arthritis is uncommonly combined. There have been a few reports that described cases of rheumatoid arthritis (RA) concurrent with MDS, but advanced rheumatoid arthritis with typical joint deformities has rarely been reported. We report a case of rheumatoid arthritis with atlantoaxial subluxation combined with refractory anemia in a 31-year-old woman.
Adult ; Arthritis, Rheumatoid/radiography ; Arthritis, Rheumatoid/pathology ; Arthritis, Rheumatoid/complications* ; Arthritis, Rheumatoid/blood ; Case Report ; Female ; Follow-Up Studies ; Human ; Myelodysplastic Syndromes/pathology ; Myelodysplastic Syndromes/complications* ; Myelodysplastic Syndromes/blood

A 39-year-old renal transplant recipient was admitted for evaluation of pancytopenia which developed gradually 10 months ago. He received renal transplantation 10 years ago and maintained relatively good renal function. Since 1 year before admission, he showed progressive decline of hematocrit, WBC count and platelet. Initial bone marrow biopsy showed erythroid hypoplasia, and second bone marrow biopsy revealed myelodysplasia. Clinical course was fatal. In conclusion, if patients with long term azathioprine treatment show progressive pancytopenia, one should suspect the possibility of myelodysplastic syndrome.
Adult ; Azathioprine* ; Biopsy ; Blood Platelets ; Bone Marrow ; Hematocrit ; Humans ; Kidney Transplantation ; Myelodysplastic Syndromes* ; Pancytopenia ; Transplantation*

OBJECTIVETo discuss the impact of comorbidities on the outcomes of patients with MDS.

METHODSThe clinical characteristics of 676 MDS patients with detailed comorbidities evaluations was analyzed retrospectively.

RESULTSThere were 395/676 cases (58.4%) with comorbidities (group 1), 281/676 cases (41.6%) without (group 2). Significant differences were seen in the distribution of age (≥ 60 y), bone marrow blasts, abnormal karyotype, WHO 2008 subtypes and IPSS-R risk cohorts (P<0.05) between the two groups. While gender, HGB concentrations, WBC levels, platelet levels and serum ferritin were not significantly different (P>0.05). Independent prognostic significance of comorbidities was seen in both uni-variate and multi-variate analyses (P<0.001). According to MDS-specific comorbidity index (MDS-CI), the median survival were 32(1-153) months, 19(2-85) months and 13(1-37) months in the low-risk, intermediate-risk and high-risk cohorts respectively, while 96(1-166) months in cohorts without any comorbidities, of which significant differences were seen (P<0.001). The MDS-CI allowed further stratification in the IPSS-R low-risk, intermediate-risk and high-risk cohorts (P<0.001).

CONCLUSIONComorbidities provides prognostic stratification independently of IPSS-R for MDS patients.

Abnormal Karyotype ; Blood Platelets ; Comorbidity ; Humans ; Leukocytes ; Myelodysplastic Syndromes ; Prognosis ; Retrospective Studies ; Risk

No abstract available.
Antilymphocyte Serum ; Humans ; Leukemia ; Myelodysplastic Syndromes ; Peripheral Blood Stem Cell Transplantation ; Tissue Donors

OBJECTIVEThrombocytopenic hemorrhage is one of the major appearance in pediatric hemorrhagic diseases, in which, idiopathic thrombocytopenic purpura (ITP) is the most common disease. Thrombocytopenia is the earliest phenomenon or the only one in certain phases of hemorrhagic diseases, such as ITP, aplastic anemia (AA) and myelodysplastic syndrome (MDS). By now, the pathogenesis of thrombocytopenia in different diseases has not been clearly determined. At present, it is very difficult to diagnose these diseases and estimate their prognosis with current clinical data. In this study, morphological characteristics and hematopoiesis function of bone marrow megakaryocyte in pediatric patients with ITP, AA and MDS were observed and the cause and mechanism of different thrombocytopenias were analyzed.

METHODSThere were 16 children with ITP, 17 with AA and 16 with MDS in this study. CD41 McAb immunohistochemical technique was used to detect micromegakaryocyte on bone marrow smears. Plasma clot culture and CD41 McAb immunohistochemical technique were used for the MK-colony forming assay. The colony formation rate of colony formation unit-megakaryocyte (CFU-MK) and burst formation unit-megakaryocyte (BFU-MK) were counted.

RESULTSThere was no statistical difference on the positive rates of micromegakaryocyte and type I lymphoid small micromegakaryocyte between groups of ITP and control. The number of micromegakaryocyte and the formation rates of CFU-MK in ITP group were significantly higher than those in control group. Among AA patients, the numbers of MK, micromegakaryocyte and the formation rates of CFU-MK, BFU-MK in vitro significantly decreased. There was no significant difference in the positive rate of micromegakaryocyte between groups of MDS and control, but the number of micromegakaryocyte and the positive rate of type I lymphoid micromegakaryocyte were significantly higher than those of control group. There was no statistical difference of the formation rate of CFU-MK between groups of MDS and control. But in 63% childhood patients, the formation rate of CFU-MK decreased, 25% increased,and 13% was normal; BFU-MK formation rate decreased significantly in MDS group.

CONCLUSIONOverproliferation of bone MKs may exist in most ITP patients. For obviating the nosogenetic factors, the normal MK releasing platelet could be easily found in the culture system. But the colony formation rate of MK decreased in a few patients with CITP. The abnormality of MK might be one of the reasons for thrombocytopenia in partial patients with ITP. Underproliferation of MKs may exist in AA, but no pathosishemogenesis was found. The dysfunction of early phase MK progenitor and stem cell might be the major reason for AA, but not the abnormality of hematopoietic microenvironment. There may be two kinds of megakaryocyte clones in bone marrow of children with MDS. One may be pathologic and potentially malignant micromegakaryocytes, the other may be the normal megakaryocytic precursors. The increase of pathologic MK resulted in abnormal development and maturation of MK in bone marrow. The change of megakaryopoiesis showed different in ITP, AA or MDS. Using bone marrow smear megakaryocyte counting, small micromegakaryocyte immunohistochemical detecting and the formation rate of bone marrow MK colony assay, the different thrombocytopenia can be diagnosed during the early stage of ITP, AA or MDS.

Adolescent ; Anemia, Aplastic ; blood ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Megakaryocytes ; pathology ; physiology ; Myelodysplastic Syndromes ; blood ; Purpura, Thrombocytopenic ; blood

OBJECTIVETo explore the application value of detection of Hepcidin together with indicator of iron overload on clinical diagnosis and treatment of MDS with iron overload by measuring Hepcidin and iron load indices of transfusion dependent myelodysplastic syndrome (MDS) patients.

METHODSEnzyme-linked immunosorbent assay (ELISA), radioimmunoassay and colorimetry were used to determine the Hepcidin, serum ferritin (SF) and serum iron (SI) levels of 106 serum samples from 68 cases of transfusion dependent MDS patients, 30 serum samples of MDS patients without transfusion and 60 serum samples of controls.

RESULTSFor MDS group, Hepcidin level in blood transfusion < 9 U subgroup was significantly higher than that in control group \[(583 ± 50) µg/L vs (175 ± 35) µg/L\] and there was a strong positive correlation between Hepcidin levels and SF (r = 0.976), but no correlation between Hepcidin and SI (r = 0.284); Both Hepcidin and SF level in transfusion 9 ∼ 24 U subgroup was significantly higher than those in control group \[(665 ± 80) µg/L vs (175 ± 35) µg/L; (1445 ± 275) µg/L vs (112 ± 26)µg/L\]; whereas for SI level, there was no difference between transfusion 9 ∼ 24 U subgroup and the control group. Hepcidin did not correlate with SF or SI; For blood transfusion > 24 U group, all of Hepcidin, SF and SI levels were higher than those in control groups \[(703 ± 64) µg/L vs (175 ± 35) µg/L; (2587 ± 352) µg/L vs (112 ± 26)µg/L; (20 ± 4) µg/L vs (14 ± 4) µmol/L\], Hepcidin negatively correlated with SF and SI (r = -0.536; r = -0.456). Hepcidin levels of RARS patients were significantly lower than RAEB patients \[(260 ± 40) µg/L vs (442 ± 51) µg/L\], and there was no significant difference between RARS group and control group regardless of the number of blood transfusion.

CONCLUSIONBoth Hepcidin and SF levels in MDS patients regardless of transfusion dependent or not, or the number of blood transfused were higher than those of normal controls, the increase of Hepcidin can not synchronize with the increase of SF level due to the increased blood transfusion, when blood transfusion > 24 U, Hepcidin level showed a negative relationship with SF and SI, reflecting the decreased ability of Hepcidin to inhibit body iron absorption during the increase of blood transfusion, which finally would lead to iron overload. We can predict the occurrence of iron overload in transfusion dependent MDS patients by dynamic monitoring concentration of Hepcidin.

Adult ; Aged ; Aged, 80 and over ; Antimicrobial Cationic Peptides ; blood ; Blood Transfusion ; Female ; Ferritins ; blood ; Hepcidins ; Humans ; Iron ; blood ; Iron Overload ; Male ; Middle Aged ; Myelodysplastic Syndromes ; blood ; therapy

OBJECTIVETo exploit the role of bone marrow (BM) and peripheral blood (PB) fluorescence in situ hybridization (FISH) in cytogenetic evaluation of myelodysplastic syndrome (MDS).

METHODSThe metaphase cytogenetics and BM interphase FISH were prospectively compared in 112 cases of de novo MDS. At the same time, comparison of BM and PB FISH was conducted in 56 cases.

RESULTSThe differences between metaphase cytogenetics and BM FISH were observed in 22 (54%) of 41 cases with clonal karyotypic abnormalities, most of differences were caused by the limitation of FISH probe panel which could not target all of the regions with aberrations. Only 6 (27%) of 22 differences were involved in our probe regions, the FISH results did not change their cytogenetic risk categories. BM FISH testing was abnormal in 15 (21%) of 71 cases with normal karyotypes, FISH testing was abnormal in 14/51 (27%) and 1/20 (5%) cases with fewer than 20 normal metaphases or more than 20 normal metaphases. Comparison of FISH results of PB and BM samples showed abnormal PB FISH results in 21 (72%) of 29 cases with abnormal BM FISH results, and in 1 (4%) of 27 cases with normal BM FISH results.

CONCLUSIONBM FISH should be used to MDS cases with fewer than 20 normal metaphases. Although PB FISH testing is limited by a relatively high false negative rate, it is a reasonable choice to cases with failure of BM aspiration.

Adolescent ; Adult ; Aged ; Female ; Humans ; In Situ Hybridization, Fluorescence ; Karyotyping ; Male ; Middle Aged ; Myelodysplastic Syndromes ; blood ; genetics ; Prospective Studies ; Young Adult

OBJECTIVETo explore the clinical features, diagnosis and treatment of pediatric myelodysplastic syndrome (MDS).

METHODSTwenty-eight children with MDS between January 2006 and March 2012 were enrolled in the study. Clinical symptoms, signs, laboratory examinations, treatment and outcomes were retrospectively studied.

RESULTSAnemia (96%), bleeding (68%), fever (68%) and hepatosplenomegaly (61%) were main clinical manifestaions in the 28 patients. Three cases (11%) converted into acute monocytic leukemia (M5), erythroleukemia (M6) or acute megakaryocytic leukemia (M7) one to two months later. Bone marrow proliferation mainly demonstrated as active or obviously active. One or two lineages of hematopoietic dysplasia were mostly observed in all 28 cases and obvious iron metabolism disorders were found in these patients. Cytogenetic abnormalities were detected in 45% of the 28 cases, most of which were numeral chromosome abnormalities. T cell, B cell and NK cell numbers decreased, Th cell numbers decreased, Ts cell numbers increased and Th /Ts inversed. Eight cases gave up treatment when confirmed. Of the 8 cases receiving symptomatic and supportive treatment alone, one was lost, one showed disease stability, and the remaining 6 cases showed disease progression. One patient who underwent induced differentiation and one who received hematopoietic therapy showed disease progression. Ten patients underwent chemotherapy. Two cases had no bone marrow remission after single agent chemotherapy. Of the 8 cases who underwent multi-drug combination chemotherapy, 4 cases achieved partial or complete remission of bone marrow.

CONCLUSIONSPediatric MDS is characterized by a lack of typical clinical manifestations, and a high rate of conversion to leukemia. Bone marrow proliferation is mainly active in children with MDS. One or two lineages of hematopoietic dysplasia is common. Among the cytogenetic abnormalities, numeral chromosome abnormalities are common. Obvious iron metabolism disorders and abnormal cellular immunity are found in children with MDS. Multi-drug combination chemotherapy appears to slow the course of the disease.

Adolescent ; Bone Marrow Examination ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Myelodysplastic Syndromes ; blood ; classification ; diagnosis ; therapy