Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report the clinical, hematologic, and cytogenetic findings for a child with secondary myelodysplastic syndrome (MDS) after treatment for a primitive neuroectodermal tumor. At the time of conversion to MDS, conventional cytogenetics revealed an unbalanced der(6)t(1;6) that resulted in trisomy of the long arm of chromosome 1 and partial monosomy and duplication of 6p. Using alpha satellite probes, fluorescence in situ hybridization of bone marrow cells showed that the rearranged chromosome contained the centromeres of both chromosomes 1 and 6, thus forming a dic(1;6) resulting in trisomy 1q. This report is the first to describe a case of childhood secondary myelodysplastic syndrome associated with a trisomy 1q involving chromosome 6.
 ...
PMID:Trisomy of the long arm of chromosome 1 resulting in a dicentric derivative (6)t(1;6) chromosome in a child with myelodysplastic syndrome following treatment for a primitive neuroectodermal tumor. 1072 89

MIC2 is characteristically expressed in lymphoblastic lesions and Ewing's/primitive neuroectodermal tumor sarcomas. Although MIC2 has recently been reported in chloroma and rare terminal deoxynucleotidyl transferase-positive acute myelogenous leukemia (AML), the incidence and the significance of MIC2 (CD99) immunoreactivity in myeloid lesions is not clear. In this study, we evaluated MIC2 positivity in a variety of myeloid diseases and normal marrow to determine its incidence and distribution in myeloid diseases; its correlation with flow cytometric and cytogenetic data in AML; and its association with leukemic transformation, relapse, and chloroma formation. Paraffin sections of 11 chloromas and 94 bone marrow core biopsies from 66 patients were stained with CD99 monoclonal antibody 12E7. Of 94 bone marrow core biopsies, there were 30 AML (fragment antigen binding M0 to M6), 23 remissions, 5 relapses, 12 myeloproliferative disorders, 13 myelodysplastic syndromes, and 11 normal marrows from patients who did not have leukemia. CD99 immunoreactivity was evaluated with light microscopy. MIC2 expression was seen in leukemic blasts in 6 of 11 chloromas (55%) and 13 of 30 AML (43%) but rarely in myeloproliferative disorders, myelodysplastic syndromes, remission, and normal marrow. CD99 tended to be positive in M1-, M3-, and HLA-Dr-negative AML and negative in AML with relapse. MIC2 expression did not correlate with the karyotype independent of French-American-British Cooperative Group classification and the disease remission or occurrence of chloroma in AML. We concluded that MIC2 is commonly expressed in leukemic blasts of AML and is not predictive of leukemic transformation from myeloproliferative disorders and myelodysplastic syndromes or chloroma formation. Caution should be taken when using MIC2 as a marker for Ewing's sarcoma/ primitive neuroectodermal tumor or lymphoblastic lymphoma on paraffin sections of either soft tissue or bone marrow specimens.
 ...
PMID:Immunoreactivity of MIC2 (CD99) in acute myelogenous leukemia and related diseases. 1078 14

This study summarizes the development and progress of the pediatric Hematopoietic Stem Cell Transplantation (HSCT) program in Puerto Rico. Data from the two pediatric transplant centers was collected from July 1994 through December 2009. Overall, in the last 15 years 97 transplants have been performed to 87 children and young adults. These included 55 males and 32 females, aged between 1 and 38 years of age. The diagnosis included: acute leukemia, chronic leukemia, myelodysplasia, aplastic anemia, histiocytosis, neuroblastoma, lymphomas, PNET, Wilms tumor, and desmoplastic round cell tumor. Sources of hematopoietic stem cells for transplantation included: 5 autologous bone marrow, 23 allogeneic bone marrow using HLA matched sibling; 18 allogeneic peripheral blood stem cell (PBSC) using HLA-matched sibling and one syngeneic PBSC; 4 haploidentical T-cell depleted bone marrow transplant and 47 autologous PBSC. The results in the past 15 years are encouraging and conform that HSCT is a valuable treatment option in our pediatric population to no other alternative therapy. Our experience compares to those published by others with a 15-year overall survival of 48%. In this same group, the transplant related mortality was 14.9% comparable to published experience in Europe and USA.
 ...
PMID:Pediatric hematopoietic stem cell transplantation: fifteen year experience in Puerto Rico. 2388 68