Abnormal chromatin clumping in granulocytes in a case of Ph1-negative, BCR-ABL rearrangement positive, haematologically atypical chronic myeloid leukaemia (CML).
In the present study, we report the case of a patient displaying an abnormal chromatin clumping (ACC) syndrome, a rare disease which shares features with both myeloproliferative and myelodysplastic disorders. Although various non specific cytogenetic abnormalities have been observed in ACC, the presence of a Ph1 chromosome has not been reported. In our patient, despite a lack of Ph1, PCR analysis of blood and bone marrow samples revealed a BCR-ABL rearrangement. These results indicate that at least some cases of ACC syndrome could represent a form of Ph1-negative chronic myeloid leukaemia.
Laboratoire d'Hematologie, Centre Hospitalier et Universitaire de Bicetre, Le Kremlin-Bicetre, France.
G
G
Tertian
M
M
Misrahi
D
D
Diallo
F
F
Mielot
C
C
Leonard
S
S
Salmeron
G
G
Tchernia
D000368:Aged; D001483:Base Sequence; D001856:Bone Marrow Examination; D002843:Chromatin / Q000648:ultrastructure*; D016044:Fusion Proteins, bcr-abl / Q000235:genetics*; D015321:Gene Rearrangement; D006098:Granulocytes / Q000648:ultrastructure*; D006801:Humans; D007621:Karyotyping; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000097:blood / Q000235:genetics* / Q000473:pathology; D054438:Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative / Q000097:blood / Q000235:genetics* / Q000473:pathology; D008297:Male; D008969:Molecular Sequence Data; D016133:Polymerase Chain Reaction; D013577:Syndrome; D014178:Translocation, Genetic
D002843:Chromatin; D016044:Fusion Proteins, bcr-abl