| Disease | ANLL and/or MDS |
| Phenotype / cell stem origin | M2, M4 ANLL, often preceded by MDS;
M1 ANLL or RAEB at times;
may be secondary to toxic exposure;
a primitive myeloid progenitor is likely to be involved |
| Epidemiology | 1% of ANLL;
found at any age, but median age (25-30 yrs) is less than usual in ANLL;
rare in the elderly;
sex ratio: 1M/1F;
blood data: marked basophilia (> 1% of nucleated cells) in one third to half of the patients |
| Cytology | TdT +; Auer rods |
| |  |
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| The translocation t(6;9)(p23;q34) results in the formation of a chimeric fusion gene: DEK (6q23) and CAN (9q34). CAN is a putative oncogene which may be activated by fusion of its 3' end to other genes than DEK. One such recently reported gene is called SET and leads to expression of a SET/CAN fusion RNA. The t(6;9)(p21-22;q34) may be seen in either AML M2 or less frequently in M4 or MDS and acute myelofibrosis often in association with excessz basophils. The t(6;9) is reported mostly in young adults. The prognosis of patients carrying the t(6;9) is unfavorable - Courtesy Georges Flandrin, CD-ROM AML/MDS G.Flandrin/ICG. TRIBVN |
|
| Prognosis | remission difficult to obtain; CR in only half cases; median survival around 1 yr |
| Increased numbers of marrow basophils may be associated with a t(6;9) in ANLL. |
| Pearson MG, Vardiman JW, Le Beau MM, Rowley JD, Schwartz S, Kerman SL, Cohen MM, Fleischman EW, Prigogina EL |
| American journal of hematology. 1985 ; 18 (4) : 393-403. |
| PMID 3976650 |
| |
| The (6;9) chromosome translocation, associated with a specific subtype of acute nonlymphocytic leukemia, leads to aberrant transcription of a target gene on 9q34. |
| von Lindern M, Poustka A, Lerach H, Grosveld G |
| Molecular and cellular biology. 1990 ; 10 (8) : 4016-4026. |
| PMID 2370860 |
| |
| Dek-can rearrangement in translocation (6;9)(p23;q34). |
| Soekarman D, von Lindern M, van der Plas DC, Selleri L, Bartram CR, Martiat P, Culligan D, Padua RA, Hasper-Voogt KP, Hagemeijer A |
| Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K. 1992 ; 6 (6) : 489-494. |
| PMID 1602786 |
| |
| Translocation t(6;9) in acute non-lymphocytic leukaemia results in the formation of a DEK-CAN fusion gene. |
| von Lindern M, Fornerod M, Soekarman N, van Baal S, Jaegle M, Hagemeijer A, Bootsma D, Grosveld G |
| Bailliere's clinical haematology. 1992 ; 5 (4) : 857-879. |
| PMID 1308167 |
| |
| Translocation t(6;9)(p23;q34) in acute myeloid leukemia without myelodysplasia or basophilia: two cases and a review of the literature. |
| Lillington DM, MacCallum PK, Lister TA, Gibbons B |
| Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K. 1993 ; 7 (4) : 527-531. |
| PMID 8464230 |
| |
| Interaction of cellular proteins with the leukemia specific fusion proteins DEK-CAN and SET-CAN and their normal counterpart, the nucleoporin CAN. |
| Fornerod M, Boer J, van Baal S, Morreau H, Grosveld G |
| Oncogene. 1996 ; 13 (8) : 1801-1808. |
| PMID 8895527 |
| |
