The World Health Organization classifies atypical chronic myeloid leukemia (aCML) as a myeloproliferative/myelodisplastic hematological disorder. The primary manifestations are leukocytosis with disgranulopoiesis, absence of basophilia and/or monocytosis, splenomegaly and absence of Philadelphia chromosome or BCR/ABL fusion. Overall 50-65% of patients demonstrate karyotypic abnormalities, although no specific cytogenetic alterations have been associated with this disease. X chromosome alterations have been rarely reported in myeloid malignancies. Although Isodicentric X, idic(X)(q13) is well known in females with myelodysplastic syndromes (MDS), little data are available on X isochromosome and its pathogenetic potential in these disorders. i(X)(p10) is observed in a variety of hematologic malignancies, both myeloid and lymphoid, as a unique abnormality, as well as part of a more complex karyotype, in females and less frequently in male patients. The present report describes the first patient with aCML, with documented isolated i(X)(p10), who developed a secondary acute myeloid leukemia (sAML).

Gurnari, C., Panetta, P., Fabiani, E., Nardone, A.m., Postorivo, D., Falconi, G., et al. (2018). Identification of i(X)(p10) as the sole molecular abnormality in atypical chronic myeloid leukemia evolved into acute myeloid leukemia. MOLECULAR AND CLINICAL ONCOLOGY, 8(3), 463-465 [10.3892/mco.2017.1543].

Identification of i(X)(p10) as the sole molecular abnormality in atypical chronic myeloid leukemia evolved into acute myeloid leukemia

Gurnari, Carmelo;Panetta, Paola;Rizzo, Manuela;Lo-Coco, Francesco;Voso, Maria Teresa
2018-03-01

Abstract

The World Health Organization classifies atypical chronic myeloid leukemia (aCML) as a myeloproliferative/myelodisplastic hematological disorder. The primary manifestations are leukocytosis with disgranulopoiesis, absence of basophilia and/or monocytosis, splenomegaly and absence of Philadelphia chromosome or BCR/ABL fusion. Overall 50-65% of patients demonstrate karyotypic abnormalities, although no specific cytogenetic alterations have been associated with this disease. X chromosome alterations have been rarely reported in myeloid malignancies. Although Isodicentric X, idic(X)(q13) is well known in females with myelodysplastic syndromes (MDS), little data are available on X isochromosome and its pathogenetic potential in these disorders. i(X)(p10) is observed in a variety of hematologic malignancies, both myeloid and lymphoid, as a unique abnormality, as well as part of a more complex karyotype, in females and less frequently in male patients. The present report describes the first patient with aCML, with documented isolated i(X)(p10), who developed a secondary acute myeloid leukemia (sAML).
mar-2018
Pubblicato
Rilevanza internazionale
Articolo
Esperti anonimi
Settore MED/15 - MALATTIE DEL SANGUE
English
atypical chronic myeloid leukemia; isochromosome(X)(p10); secondary acute myeloid leukemia
Gurnari, C., Panetta, P., Fabiani, E., Nardone, A.m., Postorivo, D., Falconi, G., et al. (2018). Identification of i(X)(p10) as the sole molecular abnormality in atypical chronic myeloid leukemia evolved into acute myeloid leukemia. MOLECULAR AND CLINICAL ONCOLOGY, 8(3), 463-465 [10.3892/mco.2017.1543].
Gurnari, C; Panetta, P; Fabiani, E; Nardone, Am; Postorivo, D; Falconi, G; Franceschini, L; Rizzo, M; Rapisarda, Vm; De Bellis, E; Lo-Coco, F; Voso, Mt
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2108/194349
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