Lymphoid blast crisis in a patient with Philadelphia-chromosome-negative chronic myelocytic leukemia.
A 24-year-old man with Philadelphia-chromosome (Ph)-negative chronic myelocytic leukemia (CML) developed lymphoid blast crisis. In the chronic phase, karyotype was normal and the clinical and hematological features were indistinguishable from those of Ph-positive CML. Rearrangement of the breakpoint cluster region (bcr) was observed. In the blast phase, blast cells showed early B-cell phenotype (CALLA+, Ia+, TdT+) with a rearranged immunoglobulin heavy-chain gene joining region (JH). By using an immunoblotting method and antiphosphotyrosine sera, P210bcr-abl protein was detected. The patient responded well to vincristine and prednisolone (VP) therapy. These findings support the concept that Ph-negative bcr+ CML can behave in a very similar fashion to Ph-positive CML, not only in the clinical features of the chronic phase but also in the manner of the blast crisis.
Takahira
H
H
Third Department of Internal Medicine, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
Shibata
K
K
Hirata
J
J
Umemura
T
T
Nishimura
J
J
Nawata
H
H
D000328:Adult; D001707:Biopsy, Needle; D001752:Blast Crisis / Q000235:genetics* / Q000276:immunology / Q000473:pathology; D001853:Bone Marrow / Q000201:enzymology / Q000473:pathology; D015326:Gene Rearrangement, B-Lymphocyte, Heavy Chain; D005803:Genes, Immunoglobulin; D006651:Histocytochemistry; D006801:Humans; D015151:Immunoblotting; D007621:Karyotyping; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000235:genetics* / Q000276:immunology / Q000473:pathology; D008297:Male; D010641:Phenotype; D010677:Philadelphia Chromosome