Nelken B, Lai J L, Facon T, Lambilliotte A, Gaudier B
L'Unité d'Hématologie Oncologique, Hôpital Calmette, Lille.
Arch Fr Pediatr. 1988 Dec;45(10):813-4.
Three new cases of monosomy 7 are described. Two children, before onset of overt leukemia, had a preleukemic state: one with thrombopathy and myelodysplastic syndrome, the other with a moderate splenomegaly and an absolute monocytosis. In these two cases the leukemia was chemoresistant. The last child had a subacute myelomonocytic syndrome (juvenile type of chronic myelogenous leukemia) without high fetal hemoglobin value. She died from cachexia. The poor prognosis of monosomy 7 is underlined and such a chromosome deletion should be searched in myeloproliferative syndrome with monocytosis.
本文描述了3例新的7号染色体单体病例。两名儿童在明显白血病发作前处于白血病前期状态:一名患有血栓形成异常和骨髓增生异常综合征,另一名有中度脾肿大和绝对单核细胞增多症。这两例白血病对化疗耐药。最后一名儿童患有亚急性粒单核细胞综合征(慢性粒细胞白血病幼年型),胎儿血红蛋白值不高。她死于恶病质。强调了7号染色体单体的预后不良,并且在单核细胞增多的骨髓增殖综合征中应检查这种染色体缺失。