i(17q) appearing in acute phase in Ph1-negative, BCR-negative CML

N. Uike, S. Yamashita, K. Obama, H. Takahira, H. Nakai, J. Inazawa, T. Umemura, K. Akashi, M. Kozuru

Research output: Contribution to journalArticle

Abstract

A 36-year-old woman was referred to our hospital because of splenomegaly in February 1989. The leukocyte count was 55,500/microliter without hiatus leukemicus. The leukocyte alkaline phosphatase score was low (29). The bone marrow showed myeloid hyperplasia (24.8% myeloblasts) but no dysplastic change. The karyotype of the bone marrow cells was 46, XX and a diagnosis of Ph1 (-) CML was made. Treatment with VCR, 6MP and prednisolone made 7-month duration chronic phase, but the abnormal karyotype.[46, XX, i(17q)] gradually increased to 100% of bone marrow cells. The patient died in June 1990. The evidence that not only a BCR rearrangement but also messages of BCR/ABL fusion gene were negative made us able to differentiate this case from Ph1(-), BCR(+) CML. The addition of an i(17q) results in partial monosomy of 17q (17q13;p53 gene) and partial trisomy of 17q (17q11.2-12;G-CSF gene). We examined the rearrangement of p53 gene and G-CSF-dependent tumor cell growth in vitro, demonstrating one allelic loss of p53 gene and independent cell growth on G-CSF respectively. It is thought that in Ph1 (-), BCR (-) CML as well as in Ph1 (+) CML, an i(17q) is related to the progression but not to the initiation of these leukemias. However the precise mechanism, including p53 gene inactivation by point mutation, is still to be elucidated.

Original languageEnglish (US)
Pages (from-to)694-699
Number of pages6
Journal[Rinshō ketsueki] The Japanese journal of clinical hematology
Volume33
Issue number5
StatePublished - May 1992

ASJC Scopus subject areas

  • Medicine(all)

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    Uike, N., Yamashita, S., Obama, K., Takahira, H., Nakai, H., Inazawa, J., Umemura, T., Akashi, K., & Kozuru, M. (1992). i(17q) appearing in acute phase in Ph1-negative, BCR-negative CML. [Rinshō ketsueki] The Japanese journal of clinical hematology, 33(5), 694-699.