Myelodysplasitic syndrome in two young brothers

Abstract

We report the youngest cases of myelodysplastic syndrome (MDS) in two brothers aged 7 and 2 years. The maternal grandfather and maternal grandmother had been exposed to radioactive fallout after the atomic bomb attack on Hiroshima in 1945. The elder brother demonstrated pancytopenia with < 1% blast cells in his peripheral blood and < 5% in his bone marrow at diagnosis. The younger brother was thrombocytopenic without increased blasts. The karyotype of bone marrow cells from the elder brother was 46,XY, -7, +der (7), t(1:7) (lqter-lq11::7q11-7pter), but the younger brother's karyotype was normal. Immature myeloid cells in the bone marrow from both brothers were morphologically abnormal. A diagnosis of refractory anaemia (RA) was made in both brothers. Atavism due to radioactive poisoning was suspected in the development of MDS in these two cases.

Cancer in relatives of children with myelodysplastic syndrome, acute and chronic myeloid leukaemia

“…Familial occurrence of myelodysplastic syndrome (MDS), including juvenile chronic myeloid leukaemia ( JCML) and monosomy 7 syndrome, has been reported in a number of cases (Randall et al, 1965;Holton & Johnson, 1968;Li et al, 1981;Kobrinsky et al, 1982;Chitambar et al, 1983;CastroMalaspina et al, 1984;Carroll et al, 1985;Paul et al, 1987;Shannon et al, 1989;Brandwein et al, 1990;Gilchrist et al, 1990;Hirose et al, 1995;Passmore et al, 1995). Many of the familial cases have been associated with monosomy 7, which is the most common cytogenetic abnormality in childhood MDS (Hasle, 1994).…”

Familial occurrence of chronic neutrophilic leukaemia

“…The occurrence of myeloproliferative diseases has been also described in atomic bomb survivors (Preston et al , 1994). Although no evidence exists of genetic effects in descendants of atomic bomb survivors to date, atavism due to radioactive poisoning has been suggested in two sibling cases of myelodysplastic syndrome whose maternal grandfather and maternal grandmother had been exposed to radioactive fallout after the atomic bomb attack (Hirose et al , 1995).…”

Familial occurrence of chronic neutrophilic leukaemia

“…The occurrence of myeloproliferative diseases has been also described in atomic bomb survivors (Preston et al, 1994). Although no evidence exists of genetic effects in descendants of atomic bomb survivors to date, atavism due to radioactive poisoning has been suggested in two sibling cases of myelodysplastic syndrome whose maternal grandfather and maternal grandmother had been exposed to radioactive fallout after the atomic bomb attack (Hirose et al, 1995). Recently, an association of CNL with a rare type of BCR/ ABL rearrangement with a breakpoint between exons e19 and e20 of the BCR gene has been reported (Pane et al, 1996).…”