We report the clinical, hematologic, cytogenetic, and molecular characteristics of 13 patients with Philadelphia-negative (Ph-), bcr-negative atypical chronic myelogenous leukemia (CML). In the majority of cases, the phenotypic features at presentation resembled those of typical CML. However, these patients presented with a higher median age, lower median hemoglobin levels, and lower leukocyte and platelet counts than patients with Ph-positive CML. Cytogenetic analysis showed an abnormal karyotype in only one case. Southern blot investigation, using probes exploring the entire M-bcr region, demonstrated the absence of genomic bcr-abl rearrangements. The assessment of clonality in five patients (study of X-methylation patterns in females heterozygous at the DXS255 locus) indicated the proliferation of a monoclonal cell population. Disease evolution was mostly characterized by bone marrow failure, extramedullary infiltrates, and poor response to chemotherapy, without evidence of overt acute transformation. Our observations suggest that some hematologic and clinical features and the modalities of disease progression are presently the most helpful factors in distinguishing these bcr/abl-negative patients from those with typical bcr+CML. The differences existing also with chronic myelomonocytic leukemia (CMMoL), allow the consideration of ph-/bcr- CML as a separate entity, the nature of which remains to be elucidated.

Montefusco, E., Alimena, G., Lo Coco, F., De Cuia, M.r., Wang, Y.z., Aloe Spiriti, M.a., et al. (1992). Ph-negative and bcr-negative atypical chronic myelogenous leukemia: biological features and clinical outcome. ANNALS OF HEMATOLOGY, 65(1), 17-21.

Ph-negative and bcr-negative atypical chronic myelogenous leukemia: biological features and clinical outcome

LO COCO, FRANCESCO;
1992-07

Abstract

We report the clinical, hematologic, cytogenetic, and molecular characteristics of 13 patients with Philadelphia-negative (Ph-), bcr-negative atypical chronic myelogenous leukemia (CML). In the majority of cases, the phenotypic features at presentation resembled those of typical CML. However, these patients presented with a higher median age, lower median hemoglobin levels, and lower leukocyte and platelet counts than patients with Ph-positive CML. Cytogenetic analysis showed an abnormal karyotype in only one case. Southern blot investigation, using probes exploring the entire M-bcr region, demonstrated the absence of genomic bcr-abl rearrangements. The assessment of clonality in five patients (study of X-methylation patterns in females heterozygous at the DXS255 locus) indicated the proliferation of a monoclonal cell population. Disease evolution was mostly characterized by bone marrow failure, extramedullary infiltrates, and poor response to chemotherapy, without evidence of overt acute transformation. Our observations suggest that some hematologic and clinical features and the modalities of disease progression are presently the most helpful factors in distinguishing these bcr/abl-negative patients from those with typical bcr+CML. The differences existing also with chronic myelomonocytic leukemia (CMMoL), allow the consideration of ph-/bcr- CML as a separate entity, the nature of which remains to be elucidated.
Pubblicato
Rilevanza internazionale
Articolo
Esperti anonimi
Settore MED/15 - Malattie del Sangue
English
Cytogenetics; DNA, Neoplasm; Humans; Leukemia, Myelogenous, Chronic, BCR-ABL Positive; Remission Induction; Survival Analysis; Translocation, Genetic; Philadelphia Chromosome
Montefusco, E., Alimena, G., Lo Coco, F., De Cuia, M.r., Wang, Y.z., Aloe Spiriti, M.a., et al. (1992). Ph-negative and bcr-negative atypical chronic myelogenous leukemia: biological features and clinical outcome. ANNALS OF HEMATOLOGY, 65(1), 17-21.
Montefusco, E; Alimena, G; LO COCO, F; De Cuia, M; Wang, Y; Aloe Spiriti, M; Mancini, F; Cedrone, M; Mancini, M; Mandelli, F
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/2108/160995
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