The Philadelphia (Ph1) chromosome is found in the majority of patients affected by chronic myelogenous leukemia (CML), being considered the hallmark of the disease, but around 5-8% of patients diagnosed as CML lack the Ph1 chromosome-negative (Ph-) CML has been discussed extensively in the literature because of its heterogeneity. However, it is now accepted that some of the Ph1-CML patients have a disease indistinguishable from Ph1-positive (Ph+) CML. It was investigated whether Ph- CML with clinical and morphological features indicating true CML would always have bcr rearrangements, as the relocation of c-abl from 9q34 into the breakpoint cluster region on 22q11 is considered a crucial event in the pathogenesis of CML. From molecular studies, it seemed that Ph- CML with features of true CML always have the bcr rearrangement, while Ph- patients, lacking such rearrangement, have atypical forms of CML. Here we describe 8 Ph- CML and myeloproliferative syndrome (MPS) patients of whom 6 were by all respects true CML cases. Nevertheless, bcr rearrangement and expression of the classic bcr/abl chimeric mRNA was found in only 1 of the 6 patients. More advanced molecular techniques will be needed to understand which molecular mechanisms underlie Ph-, bcr- CML, resulting in phenotypes sometimes indistinguishable from Ph+, bcr+ CML.

Chronic myelogenous leukemia with typical clinical and morphological features can be Philadelphia chromosome negative and "bcr negative" / Selleri, L; Emilia, Giovanni; Luppi, Mario; Temperani, Paola; Zucchini, Patrizia; Tagliafico, Enrico; Artusi, Tullio; Sarti, M; Donelli, A; Castoldi, Gl. - In: HEMATOLOGIC PATHOLOGY. - ISSN 0886-0238. - STAMPA. - 4(2):(1990), pp. 67-77.

Chronic myelogenous leukemia with typical clinical and morphological features can be Philadelphia chromosome negative and "bcr negative".

EMILIA, Giovanni;LUPPI, Mario;TEMPERANI, Paola;ZUCCHINI, Patrizia;TAGLIAFICO, Enrico;ARTUSI, Tullio;
1990

Abstract

The Philadelphia (Ph1) chromosome is found in the majority of patients affected by chronic myelogenous leukemia (CML), being considered the hallmark of the disease, but around 5-8% of patients diagnosed as CML lack the Ph1 chromosome-negative (Ph-) CML has been discussed extensively in the literature because of its heterogeneity. However, it is now accepted that some of the Ph1-CML patients have a disease indistinguishable from Ph1-positive (Ph+) CML. It was investigated whether Ph- CML with clinical and morphological features indicating true CML would always have bcr rearrangements, as the relocation of c-abl from 9q34 into the breakpoint cluster region on 22q11 is considered a crucial event in the pathogenesis of CML. From molecular studies, it seemed that Ph- CML with features of true CML always have the bcr rearrangement, while Ph- patients, lacking such rearrangement, have atypical forms of CML. Here we describe 8 Ph- CML and myeloproliferative syndrome (MPS) patients of whom 6 were by all respects true CML cases. Nevertheless, bcr rearrangement and expression of the classic bcr/abl chimeric mRNA was found in only 1 of the 6 patients. More advanced molecular techniques will be needed to understand which molecular mechanisms underlie Ph-, bcr- CML, resulting in phenotypes sometimes indistinguishable from Ph+, bcr+ CML.
4(2)
67
77
Chronic myelogenous leukemia with typical clinical and morphological features can be Philadelphia chromosome negative and "bcr negative" / Selleri, L; Emilia, Giovanni; Luppi, Mario; Temperani, Paola; Zucchini, Patrizia; Tagliafico, Enrico; Artusi, Tullio; Sarti, M; Donelli, A; Castoldi, Gl. - In: HEMATOLOGIC PATHOLOGY. - ISSN 0886-0238. - STAMPA. - 4(2):(1990), pp. 67-77.
Selleri, L; Emilia, Giovanni; Luppi, Mario; Temperani, Paola; Zucchini, Patrizia; Tagliafico, Enrico; Artusi, Tullio; Sarti, M; Donelli, A; Castoldi, Gl
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11380/613066
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