Trisomy Disorders

Trisomy 19

Posted on: October 30, 2008

Clnics and Pathology

Disease

Trisomy 19 (+19) as a sole karyotypic aberration is strongly associated with myeloid disorder. In a previously published literature review, among 31 patients with isolated +19, 25 were diagnosed with myeloid malignancy, including acute myeloid leukaemia (AML) in 14 cases and myelodysplastic syndrome (MDS) in 11 cases. Four out of the 14 AML patients had a preceding MDS phase, with +19 appearing at the time of leukaemic transformation. None of the MDS or AML cases, however, had a history of exposure to radiotherapy and chemotherapy. Hence isolated +19 is associated with a subgroup of de novo myeloid disorder, in which the clinical characteristics and prognostic impact require further delineation.

As a secondary or additional abnormality, +19 is frequently encountered in chronic myeloid leukaemia (CML). Though not as common as trisomy 8, i(17q) and extra Ph chromosome, +19 is nevertheless seen in up to 15% of CML patients with additional abnormalities.

Frequent gain of chromosome 19 or 19q was recently detected by comparative genomic hybridization in 4 out of 12 (33.3%) patients samples of acute megakaryoblastic leukaemia (AML-M7) and 9 out of 11 (81.8%) megakaryoblastic cell lines. In none of the primary patient samples was the abnormality detected by G-banding analysis. In another study on childhood and adult AML-M7, +19 was detected in 7 out of 53 patients, although as an additional abnormality in all cases. It appears +19 may play a role in the pathogenesis of megakaryoblastic leukaemia.

Etiology

Isolated +19 is probably associated with de novo myeloid disorders, as none of the AML and MDS cases with this abnormality reported had a history of prior radiotherapy or chemotherapy exposure.

Prognosis

Although isolated +19 is strongly associated with myeloid disorders, most probably de novo disease, its prognostic significance requires further elucidation.

 

Biobliography

Trisomy 19 in a patient with myelodysplastic syndrome and thrombocytosis. Humphries JE, Wheby MS. Cancer Genet Cytogenet 1990; 44: 187-191.

Medline 2297677

Trisomy 19 as the sole chromosomal anomaly in hematologic neoplasms. Johansson B, Billstrom R, Mauritzson N, Mitelman F. Cancer Genet Cytogenet 1994; 74: 62-65. Medline 8194050  

Frequent gain of chromosome 19 in megakaryoblastic leukemias detected by comparative genomic hybridization. Alvarez S, MacGrogan D, Calasanz MJ, Nimer SD, Jhanwar SC. Genes Chromosomes Cancer 2001; 32: 285-293. Medline 11579469

Chromosome 19 abnormalities are commonly seen in AML, M7. Nimer SD, MacGrogan D, Jhanwar S, Alvarez S. Blood 2002; 100: 3838. Medline 12411327

Contributors  Edmond S.K. Ma. Thomas S.K. Wan

Written 06/2003

InfoBioGen

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See Also:

Trisomy 19 Mosaicism

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