| Clinics and Pathology |
| Disease | myeloid lineage |
| Phenotype / cell stem origin | atypical chronic myeloid leukemia (CML), Philadelphia negative, bcr-abl negative with eosinophilia |
| Epidemiology | only two cases available |
| Clinics | massive splenomegaly in one case; some clinical features of accelerated CML |
| Cytology | peripheral blood leukoerythroblastosis; bone marrow granulocytic hyperplasia, bone marrow fibrosis (grade III-IV reticulin), dry tap |
| Pathology | extramedullary hemopoiesis in the spleen |
| Treatment | control of disease by hydroxyurea in both cases |
| Cytogenetics |
| Cytogenetics Morphological | cytogenetic result: add(5q), del(10q) |
| Probes | cosB for PDGFBR (5q33); PAC29F6 for H4/D10S170 (10q21) |
| Genes involved and Proteins |
| Gene Name | PDGFRB (Platelet Derived Growth Factor Receptor Beta) |
| Location | 5q33 |
| Protein | transmembrane and tyrosine kinase domains |
| Gene Name | H4(D10S170) |
| Location | 10q21 |
| Protein | carboxyterminal putative SH3 binding site; cytoskeletal protein? |
| Result of the chromosomal anomaly |
| Transcript | H4-PDGFBR chimeric RNA constantly present |
| External links |
| Other database | t(5;10)(q33;q21) | Mitelman database (CGAP - NCBI) | |
| Other database | t(5;10)(q33;q21) | CancerChromosomes (NCBI) |
| To be noted |
| Case Report | A case of myeloproliferative disorder with t(5;10)(q33;q21.2) case |
| Bibliography |
| - |
| Anastasiadou E et al |
| Blood. 1999 ; 94 (supplement Suppl.1) : page 51. |
| New reciprocal translocation t(5;10)(q33;q22) associated with atypical chronic myeloid leukemia. |
| Siena S, Sammarelli G, Grimoldi MG, Schiavo R, Nozza A, Roncalli M, Mecucci C, Santoro A, Carlo-Stella C |
| Haematologica. 1999 ; 84 (4) : 369-372. |
| PMID 10190953 |
| Fusion of H4/D10S170 to the platelet-derived growth factor receptor beta in BCR-ABL-negative myeloproliferative disorders with a t(5;10)(q33;q21). |
| Kulkarni S, Heath C, Parker S, Chase A, Iqbal S, Pocock CF, Kaeda J, Cwynarski K, Goldman JM, Cross NC |
| Cancer research. 2000 ; 60 (13) : 3592-3598. |
| PMID 10910073 |
| Contributor(s) |
| Written | 01-2001 | Cristina Mecucci |
| Hematology, Policlinico Monteluce, Via Brunamonti, 06123 Perugia, Italy |
| Citation |
| This paper should be referenced as such : |
| Mecucci C . t(5;10)(q33;q21). Atlas Genet Cytogenet Oncol Haematol. January 2001 . URL : http://AtlasGeneticsOncology.org/Genes/t510ID1166.html |
| © Atlas of Genetics and Cytogenetics in Oncology and Haematology | indexed on : Wed Sep 24 21:07:41 2008 |
For comments and suggestions or contributions, please contact us