Familial polycythemia vera in father and daughter.
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Biomedical subjects
Publications and source records attributed to T Sumikuma.
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A 48-year-old Japanese man was admitted to our hospital because of general fatigue, nasal bleeding, and petechiae on his extremities. He was diagnosed with acute myelomonocytic leukemia with trilineage myelodysplasia (T-MDS). Chromosomal analysis of bone marrow cells revealed t(7;11)(p15;p15), which has been rarely reported but known to be characteristic of Japanese patients. Although t(7;11)(p15;p15) has been reported mainly in acute myelogenous leukemia (AML), it can be occasionally found in so-called stem cell diseases such as chronic myelogenous leukemia or chronic myeloproliferative disorders. Therefore, t(7;11)(p15;p15) might affect trilineage progenitors or stem cells as well as myeloid lineage cells, subsequently resulting in AML with T-MDS, as in our case reported here.
Three cases of secondary acute myeloid leukemia (AML) that developed after long term treatment with oral etoposide were reported. Case 1 was a 72-year-old male in whom small cell lung cancer was diagnosed in January 1987. He developed AML (M4) in February 1993 after long-term treatment with oral etoposide (total dose 14,650 mg) t(9; 11) (p21; q23) with rearrangement of MLL genes was recognized. Case 2 was a 68-year-old female non-Hodgkin's lymphoma (NHL) was diagnosed in February 1989. AML (M4Eo) with inv(16) (p13q22) developed in March 1994 after long-term treatment with oral etoposide (total dose 5,100 mg). Case 3 was a 39-year-old male in whom NHL was diagnosed in January 1991. He developed AML(M2) with t(11; 19) (q23; p13) in May 1994 after long-term treatment with oral etoposide (total dose 20,450 mg). These three cases suggest that long-term treatment with oral etoposide may be associated with a risk of developing a secondary AML in patient with malignancies.
We immunophenotyped 128 patients with B-cell non-Hodgkin's lymphoma (B-NHL) of various histological subtypes using two-color flow cytometry (FCM), and found that lymphoma cells obtained from 31 patients (24.2%) coexpressed at least one of the following T-cell associated antigens (T-Ag); CD2 (2.3%), CD5 (18.0%) or CD7 (6.3%). Moreover, 3 patients expressed two kinds of T-Ag (CD2/CD5, CD2/CD7 or CD5/CD7) as reported by other investigators. Though we could not find coexpression of CD3, CD4 or CD8 antigen in any patients analyzed in our study, such T-Ag(+) B-NHL have also been reported in the literature. As clinical features, extranodular involvement and higher International Prognostic Index (high and high intermediate) seemed more frequent in T-Ag(+) B-NHL than T-Ag(-) B-NHL in our study. Such prognostic significance of T-Ag expression is also reported by other investigators especially in CD5(+) diffuse large B-cell lymphoma. In addition, two-color FCM for detecting such aberrant T-Ag expression in B-NHL is useful for monitoring the minimal residual disease in the subgroup with T-Ag(+) B-NHL.