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[唐氏综合征患儿短暂性异常髓系造血继之以急性白血病]

[Transient abnormal myelopoiesis followed by acute leukemia in children with Down syndrome].

作者信息

Hatae Y, Takeda T, Nakadate H, Hatayama Y, Kishino T, Ogawa Y

机构信息

Department of Pediatrics, National Sapporo Hospital, Hokkaido Cancer Center.

出版信息

Rinsho Ketsueki. 1990 Dec;31(12):1939-44.

PMID:2150419
Abstract

This report describes three cases with Down's syndrome. These cases initially had transient abnormal myelopoiesis (TAM), from which they recovered spontaneously. They finally developed into overt acute leukemia characterized by an increase of blasts, hepatosplenomegaly, and elevated lactic dehydrogenase. Of these three cases, one was thought to have ANLL, which broke out 5 months after spontaneous remission. The other two had ALL, each occurring 8 and 9 years later. Chromosomal abnormality, in addition to trisomy 21, was detected in blast cells from one of the patients with acute leukemia. All three patients with acute leukemia experienced complete remission. However, two of the three patients relapsed and died. It is noted in the literature that remission is permanent in most cases of TAM, and is rarely terminated by leukemic relapse. In view of our observations, the importance of following up on such patients who evidence apparent remission of their leukemia-like disorder is emphasized.

摘要

本报告描述了3例唐氏综合征患者。这些病例最初患有短暂性异常髓系造血(TAM),随后自发恢复。最终,他们发展为明显的急性白血病,其特征为原始细胞增多、肝脾肿大和乳酸脱氢酶升高。在这3例病例中,1例被认为患有急性非淋巴细胞白血病(ANLL),在自发缓解5个月后发病。另外2例患有急性淋巴细胞白血病(ALL),分别在8年和9年后发病。在其中1例急性白血病患者的原始细胞中,除了21三体综合征外,还检测到了染色体异常。所有3例急性白血病患者均实现完全缓解。然而,3例患者中有2例复发并死亡。文献中指出,在大多数TAM病例中缓解是永久性的,很少因白血病复发而终止。鉴于我们的观察结果,强调了对这类白血病样疾病明显缓解的患者进行随访的重要性。

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