[A case of de novo acute basophilic leukaemia: diagnostic criteria and review of the literature].
We report a case of a de novo acute basophilic leukaemia, revealed by an infectious pneumopathy in a 73 year old man. The full blood count revealed an hyperleucocytosis associated with an unregenerative normocytic normochrom anaemia and a thrombocytopenia. The blood and bone marrow smears showed a mixture of undifferentiated blast cells and basophiloblasts (high nucleo-cytoplasmic ratio, coarse basophilic cytoplasmic granules), along with basophilic precursors and basophilic polymorphonuclears. All the blasts were MPO negative but positive for the toluidine blue metachromatic coloration, which is considered as consistent with basophilic lineage. Immunophenotypic studies showed myeloid blasts, without maturity marker, CD 117 negative and CD203 cytoplasmic positive, the latter known to be highly representative of the basophilic lineage. This very clear-cut phenotype, associated with the morphology of cells, were arguments to ascertain the basophilic lineage of the blasts without the need of electron microscopic study. Cytogenetic and RNA analysis revealed the presence of a Philadelphia chromosome and of a BCR-ABL transcript with the unusual junction e6a2. Thus, imatinib was added to the conventional chimiotherapy and the patient is currently in complete remission. This clinical prompted allows us to review the literature on acute basophilic leukaemia and to state on the different diagnostic criteria of this rare disorder.
Staal-Viliare
A
A
Laboratoire d'Hématologie et de Génétique, Hôpital de Metz, CHR Metz-Thionville.
Latger-Cannard
V
V
Rault
J P
JP
Didion
J
J
Grégoire
M J
MJ
Bologna
S
S
Witz
B
B
Jonveaux
P
P
Lecompte
T
T
Rio
Y
Y
D000368:Aged; D006801:Humans; D016130:Immunophenotyping; D015471:Leukemia, Basophilic, Acute / Q000097:blood* / Q000175:diagnosis* / Q000235:genetics / Q000276:immunology; D008297:Male; D010677:Philadelphia Chromosome