| Autor: |
Yoriko Inoue, Masao Tomonaga, Masako Iwanaga, Itsuro Jinnai, Tatsuro Joh, Kengo Fuchigami |
| Rok vydání: |
2003 |
| Předmět: |
|
| Zdroj: |
Paroxysmal Nocturnal Hemoglobinuria and Related Disorders ISBN: 9784431680048 |
| Popis: |
We have detected PNH clone in four (10%) out of 40 cases of primary MDS by flowcytomery using monoclonal antibodies against CD59 and CD5ai]5._All of four cases had PIG-A mutation(s) that were heterogeneous and similar to those reported in de novo PNH and aplastic anemia/PNH. Two cases of MDS/PNH responded to immunosuppressive therapy (IST) with cyclosporin A; the PNH clone persisted even after obtaining a good response. Clonal analysis for one case of MDS/PNH with 8 trisomy by combining FACS cell-sorting and FISH revealed that the PNH clone had developed from MDS clone. Another clonal analysis in one case of AML M6 transformed from de novo PNH also revealed that M6 had originated from the PNH clone. Thus, our study indicates that (1) PNH clone can develop from MDS, (2) MDS/PNH case can respond to immunosuppressive therapy and (3) MDS/AML can originate from de novo PNH clone. However, recent literature suggests that MDS/AML clone develops not only from a PNH clone as in our cases, but also from a residual stem cell in a minority of PNH cases. Therefore, molecular genetic studies are essential to compare stem cells of PNH clone and those of non-PNH clone in respect of immune surveillance and leukemogenesis. |
| Databáze: |
OpenAIRE |
| Externí odkaz: |
|
