Klinefelter's syndrome and acute non-lymphocytic leukemia

Abstract

In five out of 51 adult, male patients with acute non-lymphocytic leukemia, Klinefelter's syndrome was demonstrated karyotypically. The incidence of this syndrome in our material is very much increased in comparison with the frequency at birth. In this article the medical histories of the patients are given and the possible connection between the constitutional chromosome abnormality and acute non-lymphocytic leukemia is discussed. Immunological or hormonal abnormalities or an increased virus susceptibility might be predisposing factors.

Cancer incidence in men with Klinefelter syndrome

“…This is in contrast to the many reports of KS associated with lymphoma (Group Fran$ais de Cytog6n6tique Hematologique, 1988;Liang et al, 1990; Koyama et al, 1992) and especialy leukaemia (Mamunes et al, 1961;Muts-Homsma et al, 1982;Gale and Toledano, 1984;Oguma et al, 1989;Adhvaryu et al, 1990;Pienkos and Meisner, 1991;Foot et al, 1992;Shaw et al, 1992). An increased risk of acute myeloid leukaemia of up to 100-fold has been reported (Muts-Homsma et al, 1982).…”

“…This is in contrast to the many reports of KS associated with lymphoma (Group Fran$ais de Cytog6n6tique Hematologique, 1988;Liang et al, 1990; Koyama et al, 1992) and especialy leukaemia (Mamunes et al, 1961;Muts-Homsma et al, 1982;Gale and Toledano, 1984;Oguma et al, 1989;Adhvaryu et al, 1990;Pienkos and Meisner, 1991;Foot et al, 1992;Shaw et al, 1992). An increased risk of acute myeloid leukaemia of up to 100-fold has been reported (Muts-Homsma et al, 1982). Major textbooks of medicine and haematology mention KS as a predisposing condition to leukaemia and lymphoma (Champlin and Golde, 1991;Nadler, 1991;Greer and Kinney, 1993 of an increased risk of leukaemia in the present study is consistent with a cytogenetic study of 1200 consecutive male patients with suspected leukaemia in which only one case of KS was found (Horsman et al, 1987 (Hasle et al, 1992).…”

Myelodysplastic Syndrome with Thrombocytosis in a Patient with Klinefelter’s Syndrome

“…Nonrandom chromo some abnormalities, such as monosomy 5 and 5q-, monosomy 7 and 7q-, trisomy 8, are commonly found in patients with these disorders [4], These facts suggest a close link between the two disorders. On the basis of cyto genetic analysis on 51 adult males with ANLL, MutsHomsma et al [3] estimated that patients with Kilnefelter's syndrome have a 100-fold increased risk of develop ing ANLL as compared to the normal population. Mukerjee et al [10] demonstrated that cells from a patient with mosaic Klinefelter's syndrome can be transformed by si mian papovavirus 40 (SV 40) with a three to tenfold higher frequency than cells from normal individuals.…”

“…These tumors were male breast cancer and mediastinal germ cell tumors which are related to hor mone-dependent tissues [1]. These patients have also been reported with a suggestion of a greater risk of hematologic malignancy such as malignant lymphoma, chronic myelo genous leukemia and acute leukemia of the lymphoid and myeloid type [2][3][4], Four cases of myelodysplastic syn drome (MDS) have previously been reported [4][5][6][7]. We describe a patient with Klinefelter's syndrome in whom re fractory anemia with excess of blasts in transformation (RAEB in T), a subtype of MDS [8], was characteristic of anemia, cosinophilia and thrombocytosis, and developed with karyotypic abnormality involving t(4;7)(q21;qll).…”

Familial Leukemia: Description of Two Kindreds and a Review of the Genetic Aspects of the Disease

“…Therefore, it was sufficiently striking to merit the few published reports by the clinicians who encountered it. These aggregates are sometimes associat ed with single gene traits such as Bloom's syndrome [2], Fanconi's anemia [3], (von Recklinghausen) neurofibro matosis [4], Wiscot-Aid rich syndrome [5], ataxia-telan giectasia [6], as well as with constitutional and familial conditions such as Down's syndrome [7], Turner's syn drome [8], Klinefelter's syndrome [9], Li-Fraumeni syn drome [10] and familial microcephaly [11], Sometimes, however, there is an excess of acute leukemia (AL) cases within blood relatives which is not associated with any of these defined conditions. Anderson [12] described 5 sib lings with leukemia whose parents were unrelated.…”