García H, Vargas L, Messen S, Pino R
Servicio de Pediatría, Hospital San Juan de Dios.
Rev Chil Pediatr. 1990 Jul-Aug;61(4):206-9.
Four cases of neonatal transient leukemoid reaction associated to Down's syndrome (TLRAD) studied in a Metropolitan Hospital at Santiago, Chile, since 1980 to 1985 are described. Diagnosis was done at the first week of live by blood cell counts in all the patients, and in every case these were characterized by high leukocyte counts (15,000 to 48,000 x mm3 blood), 13 to 25% circulating white blood cell blasts, 25 to 61% blast forms in bone marrow aspirates and normal erythrocyte and blood platelet series. In three cases the lower inferior hepatic border was palpated 3 to 5 cm under the rib's margin at the clavicular mid line. White blood cell blasts disappeared from circulation after 1 to 4 months in coincidence with reduction of liver's size to normal. Two of these patients died after remission of their leukemoid reaction as a result of serious systemic infections, without clinical nor laboratory evidence of leukemoid reaction neither leukemia, even though one of them was submitted to a complete postmortem examination. The other two cases had favourable clinical evolution and their hematologic controls have being normal along the following three years.
本文描述了1980年至1985年期间在智利圣地亚哥一家大都会医院研究的4例与唐氏综合征相关的新生儿短暂类白血病反应(TLRAD)病例。所有患者均在出生后第一周通过血细胞计数进行诊断,每例患者的特征均为白细胞计数高(每立方毫米血液中白细胞计数为15,000至48,000个),循环白细胞中有13%至25%为原始细胞,骨髓穿刺物中原始细胞形态占25%至61%,红细胞和血小板系列正常。3例患者在锁骨中线处肋缘下3至5厘米处可触及肝脏下缘。1至4个月后,循环中的白细胞原始细胞消失,同时肝脏大小恢复正常。其中2例患者在类白血病反应缓解后因严重全身感染死亡,无类白血病反应或白血病的临床及实验室证据,尽管其中1例进行了完整的尸检。另外2例患者临床病程良好,在随后的三年中血液学检查一直正常。