No abstract available.
Adolescent ; Anemia, Aplastic/*diagnosis/pathology ; Bone Marrow/pathology ; Child ; Child, Preschool ; Diagnosis, Differential ; Female ; Half-Life ; Humans ; Immunohistochemistry ; Male ; Mutation ; Myelodysplastic Syndromes/*diagnosis/pathology ; Retrospective Studies ; Tumor Suppressor Protein p53/genetics/*metabolism

Endoscopic submucosal dissection (ESD) has been successfully performed in thrombocytopenic conditions such as in patients with liver cirrhosis but successful ESD for early gastric cancer (EGC) in hematologic diseases has rarely been reported. A 52-year-old male patient, who had previously been diagnosed with myelodysplastic syndrome 2 years ago, was admitted to our hospital for ESD of EGC. ESD was performed successfully in this patient after platelet concentrates transfusion on the day of ESD. ESD might be an option for the treatment of EGC in thrombocytopenia due to hematologic diseases when optimal supportive managements are applied.
Early Detection of Cancer ; Endosonography ; Gastric Mucosa/*surgery ; Gastroscopy ; Humans ; Male ; Middle Aged ; Myelodysplastic Syndromes/complications/*diagnosis/pathology ; Stomach Neoplasms/complications/*diagnosis/pathology ; Tomography, X-Ray Computed

No abstract available.
Aged ; Base Sequence ; Bone Marrow/metabolism/pathology ; Chromosome Aberrations ; DNA/chemistry/genetics/metabolism ; Fusion Proteins, bcr-abl/*genetics ; Humans ; Immunophenotyping ; In Situ Hybridization, Fluorescence ; Male ; Myelodysplastic Syndromes/diagnosis/*genetics ; Real-Time Polymerase Chain Reaction ; Sequence Analysis, DNA

BACKGROUND: The presence of significant dysplasia in bone marrow (BM) aspirates helps to distinguish between hypocellular myelodysplastic syndrome (hMDS) and aplastic anemia (AA). Occasionally, diluted BM aspirates make it difficult to recognize dysplastic changes and can also negatively affect the detection of cytogenetic abnormalities in hMDS. We evaluated the usefulness of CD34 and p53 immunoreactivity for discriminating between hMDS and AA and for estimating survival outcomes in hMDS patients. METHODS: BM clot section (BMC) or BM biopsy (BMB) specimens were obtained from 64 hMDS/AA patients (33 with hMDS and 31 with AA) and seven controls. Immunohistochemical (IHC) staining for CD34 and p53 was performed by using the EnVision detection system (Dako, Denmark). We compared the results of IHC staining, BM findings, and chromosomal analyses, and determined overall survival outcomes. RESULTS: The number of CD34- and p53-positive BM cells was higher among the patients with hMDS than among the patients with AA (P<0.001 and P=0.001, respectively). hMDS patients with increased CD34-positive cells had significantly poorer survival outcomes compared with those with normal number of CD34-positive cells (P=0.013). CONCLUSIONS: CD34 and p53 IHC stains of BMC or BMB provide useful information for differentiating between hMDS and AA. CD34 IHC staining of BMC or BMB also provides useful information for estimating survival outcomes in hMDS patients.
Adolescent ; Adult ; Anemia, Aplastic/*diagnosis ; Antigens, CD34/*metabolism ; Bone Marrow/metabolism/*pathology ; Child ; Chromosome Aberrations ; Diagnosis, Differential ; Female ; Humans ; Immunohistochemistry ; Kaplan-Meier Estimate ; Male ; Middle Aged ; Myelodysplastic Syndromes/*diagnosis/mortality ; ROC Curve ; Tumor Suppressor Protein p53/*metabolism

OBJECTIVETo explore the clinical characteristics and histopathological morphology features of bone marrow biopsies between refractory cytopenia of children (RCC) and acquired aplastic anemia (AAA) to facilitate the diagnosis, differential diagnosis and treatment of RCC and AAA.

METHODSWe retrospectively analyzed clinical data and histopathological morphology of bone marrow biopsies in RCC or AAA patients referred to our hospital from January 2011 to December 2012.

RESULTSThere were totally 130 patients studied. The final diagnoses of them were RCC in 78 cases (60.0%) and AAA in 52 cases (40.0%). The median WBC count, absolute neutrophil count, blood platelet count, hemoglobin level, and reticulocyte count were all higher in RCC children than AAA (P<0.01). All of RCC patients showed hypocellular biopsy specimens, and 84.6% (66/78) of them had cellularity of bone marrow biopsy specimens ranging from 20% to 60%. Patchy pattern distribution was seen in 98.7% (77/78) of RCC cases, and micromegakaryocyte was found in 61.5% (48/78) of RCC cases. All of AAA patients showed severe hypocellular biopsy specimens, and 88.5% (46/52) of them had cellularity of bone marrow biopsy specimens under 5%. Megakaryocyte was not found in 98.1% (51/52) of AAA cases. The response rates of immunosuppressive therapy using CsA ± rabbit anti- thymocyte globulin ± androgen ± traditional Chinese medicine for patients with RCC and AAA were 59.5% and 26.9% at 3 months (P=0.011), and 75.0% and 38.1% at 6 months, respectively (P=0.007).

CONCLUSIONRCC patients showed milder cytopenia and bone marrow hyperplasia than AAA. Patchy distribution of hematopoietic cells, erythroid islands with a marked left shift and micromegakaryocytes were decisive histomorphological patterns used to separate RCC from SAA. Immunosuppressive therapy using CsA ± rabbit anti- thymocyte globulin ± androgen ± traditional chinese medicine was an effective therapy in patients with RCC and AAA, and the outcome of immunosuppressive therapy for RCC patients was superior to that of AAA patients.

Adolescent ; Anemia, Aplastic ; diagnosis ; pathology ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Myelodysplastic Syndromes ; diagnosis ; pathology ; Retrospective Studies

Isolated deletion of the long arm of chromosome 20 [del(20q12)] is a rare abnormality in patients with de novo myelodysplastic syndrome. It is characterised by refractory thrombocytopenia, minimal haematological dysplasia and a lower risk for progression to acute myeloid leukaemia. Its distinction from chronic autoimmune thrombocytopenia, although clinically and morphologically difficult, is critical. We report a case of refractory cytopenia and unilineage dysplasia in an elderly woman with isolated del(20q12), identified via fluorescence in situ hybridisation analysis of her bone marrow. In order to avoid a misdiagnosis, we suggest that cytogenetic analysis be performed on all patients suspected to have myelodysplastic syndrome with predominant thrombocytopenic presentation.
Aged ; Biopsy, Needle ; Bone Marrow Cells ; pathology ; Chromosome Deletion ; Chromosomes, Human, Pair 20 ; Female ; Flow Cytometry ; Humans ; In Situ Hybridization, Fluorescence ; Myelodysplastic Syndromes ; diagnosis ; genetics

This study was purpose to investigate the diagnostic value of hematopoietic cell dysplasia in myelodysplastic syndrome (MDS). Sixty-four cases of WHO-MDS were detected by cytomorphology, cytohistochemical staining and bone marrow biopsy. The characteristics of hematopoietic cell dysplasia were analyzed, and its sensitivity and specificity were evaluated for WHO-MDS diagnosis. The results showed that though myeloblast, megakaryocytes presented in peripheral blood and granular Auer body, abnormal granular pseudo Pelger-Huër, vacuole of erythroid, micro-megakaryocytes appeared in bone marrow for diagnosis sensitivity were not very high, and respectively were 34.4%, 3.1%, 3.1%, 75.0%, 6.3%, 42.4%, the specificity of these characteristics was 100%. Moreover, erythroid odd nucleus, nuclear deformity, fragmentation, nuclear budding, ring sideroblasts, single and more round nuclear megakaryocyte had better reference value for WHO-MDS diagnosis. By bone marrow biopsy, the dysplasia and abnormal localization of immature precursor (ALIP) also were found in patients with WHO-MDS. More than half patients with WHO-MDS had mild to moderate increase in reticulin fibres. It is concluded that the cytomorphology assay is the base and key for the diagnosis of WHO-MDS. Diagnostic accuracy can be improved by combinative use of a variety of detection methods.
Adult ; Aged ; Aged, 80 and over ; Bone Marrow ; pathology ; Erythroid Precursor Cells ; pathology ; Female ; Granulocyte Precursor Cells ; pathology ; Humans ; Male ; Megakaryocytes ; pathology ; Middle Aged ; Myelodysplastic Syndromes ; blood ; diagnosis ; pathology ; Sensitivity and Specificity

This study was purposed to explore the correlation of chromosome karyotype with dyshaematopoiesis and reticulin in myelodysplastic syndrome (MDS). The data of 202 MDS patients diagnosed and treated in the First Affiliated Hospital of Zhengzhou University were retrospectively analyzed in term of chromosome karyotype, dyshaematopoiesis and reticulin detection results. The chromosome karyotypes were categorized according to the International Prognostic Scoring System (IPSS). The results showed that there was a positive correlation between chromosome karyotype grading and number of lineages with dyshaematopoiesis (r = 0.443, P < 0.05). The detected rates of multilineage dyshaematopoiesis in patients with good, intermediate and poor chromosome karyotypes were 44.4%, 71.4% and 96.3% respectively. There was a positive correlation between chromosome karyotype grading and reticulin grading (r = 0.451, P < 0.05). The positive rates of reticulin in patients with good grading, intermediate and poor chromosome karyotypes were 36.8%, 64.3% and 92.6% respectively. The detected rate of multilineage dyshaematopoiesis, number of lineages with dyshaematopoiesis, the positive rate of reticulin and reticulin grade in patients with poor karyotypes were higher than those in patients with intermediate or good chromosome karyotypes (separately P < 0.01). The above data in patients with intermediate chromosome karyotypes were higher than those in patients with good chromosome karyotypes (separately P < 0.01). It is concluded that the chromosome karyotype grading positively correlates with the number of lineages with dyshaematopoiesis and reticulin grading. When the chromosome karyotype changed from good to poor, the detected rate of multilineage dyshaematopoiesis, number of lineages with dyshaematopoiesis, positive rate of reticulin and reticulin grading became higher and higher.
Adolescent ; Adult ; Aged ; Bone Marrow Examination ; Female ; Humans ; Karyotype ; Karyotyping ; Male ; Middle Aged ; Myelodysplastic Syndromes ; diagnosis ; genetics ; pathology ; Reticulin ; analysis ; Retrospective Studies ; Young Adult

Myelodysplastic syndrome (MDS) is a heterogeneous disease characterized by dysplasia and ineffective hematopoiesis. The dysplasia is crucial in the diagnosis of MDS, but the morphologic abnormalities of bone marrow cells are not specific for MDS. When the morphological evaluation of marrow dysplasia and cytogenetics can not give enough informations, for diagnosis of MDS, the application of flow cytometry (FCM) for immunophenotyping in MDS will become particularly important. Multiparametric evaluation of myeloid, monocytic maturation and antigen expression pattern contribute to the identification of two or more aberrancies in MDS cases. FCM evaluation of erythroid dysplasia is particularly difficult, because of the limited availability of specific markers. By analyzing the proteins involved in cellular iron metabolism, MDS erythroid cells present an "iron-loaded" phenotype characterized by increased ferritin contents and reduced transferrin receptor, which reflects the degree of dysplasia assessed by morphology. The proportion of CD34(+) cells increased, abnormal expression of surface antigen is also important. The application of flow cytometry in detecting dysplasia of myelodysplastic syndrome is discussed in this article.
Bone Marrow Cells ; pathology ; Erythroid Cells ; metabolism ; Flow Cytometry ; Humans ; Myelodysplastic Syndromes ; blood ; diagnosis ; pathology ; Receptors, Transferrin ; metabolism

Transformation of MDS into ALL during childhood is extremely rare. We report a rare case of an 8-yr-old girl who presented with refractory cytopenia of childhood (RCC) that transformed into ALL only 3 months after the diagnosis of childhood MDS. Although no cytogenetic abnormalities were observed in conventional karyotype and FISH analysis, we found several deletions on chromosomes 5q, 12q, 13q, and 22q. Partial homozygous deletion of the RB1 gene was observed on microarray analysis, with the bone marrow specimen diagnosed as ALL. This is the first case report of transformation of ALL from childhood MDS in Korea. We also compared the clinical, cytological, and cytogenetic features of 4 previously reported childhood MDS cases that transformed into ALL.
Bone Marrow Cells/pathology ; *Cell Transformation, Neoplastic/genetics ; Child ; Chromosome Aberrations ; Female ; Gene Deletion ; Humans ; In Situ Hybridization, Fluorescence ; Karyotyping ; Myelodysplastic Syndromes/*diagnosis/genetics ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/*diagnosis/genetics ; Retinoblastoma Protein/genetics