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一名成年初发表现为血尿和双侧肾脏肿大的伴t(1;19)的前体B细胞急性淋巴细胞白血病:病例报告及文献复习

Pre-B acute lymphoblastic leukemia with t(1;19) in an adult initially presenting as hematuria and bilateral renal enlargement: a case report and literature review.

作者信息

Wu Jian, Pi Xiao-Ling, Ye Zhi-Bin

机构信息

Department of Pathology.

Department of Nephrology, Gongli Hospital affiliated to Second Military, Medical University, and.

出版信息

Clin Nephrol Case Stud. 2017 Sep 15;5:60-65. doi: 10.5414/CNCS109113. eCollection 2017.

Abstract

Although pre-B acute lymphoblastic leukemia (ALL) is the most common type of renal leukemic infiltration; the renal infiltration with leukemia cells as the initial manifestation of leukemia is very rare. Translocation (1;19)(q23;p13) is one of the most common chromosomal abnormalities in patients with ALL and is observed in 5 - 6% of children with pre-B ALL. However, the incidence of t(1;19) in adults is lower, not exceeding 3%, and the prognosis of adult patients is usually poor. Herein, we report a 52-year-old female patient with pre-B ALL who initially presented as bilateral renal enlargement. The cytogenetic analysis revealed chromosomal abnormalities including t(1;19). The patient underwent three consecutive courses of chemotherapy with VDLP (vincristine, daunorubicin, L-asp, and prednisolone) and gained a short complete remission. Her kidneys recovered to normal size, and renal function returned to normal level. However, after complete remission for only 3 months, the patient exhibited resistance to consolidation chemotherapy and indicated evidence of marrow relapse. Although we increased the drug dosage and attempted to use a different protocol, she died of severe anemia and hemorrhage almost 10 months after she was first admitted. In conclusion, pre-B cell ALL is the most common type of leukemia to present with renal infiltration as the presenting sign. Because of the poor outcome of ALL, some new therapeutic approaches may improve the patients' conditions.

摘要

尽管前体B细胞急性淋巴细胞白血病(ALL)是肾白血病浸润最常见的类型;但以白血病细胞肾浸润作为白血病首发表现的情况非常罕见。易位(1;19)(q23;p13)是ALL患者中最常见的染色体异常之一,在5%-6%的前体B细胞ALL儿童中可见。然而,成人中t(1;19)的发生率较低,不超过3%,且成年患者的预后通常较差。在此,我们报告一名52岁的前体B细胞ALL女性患者,最初表现为双侧肾脏肿大。细胞遗传学分析显示存在包括t(1;19)在内的染色体异常。该患者接受了连续三个疗程的VDLP(长春新碱、柔红霉素、L-天冬酰胺酶和泼尼松龙)化疗,并获得了短期完全缓解。她的肾脏恢复到正常大小,肾功能恢复到正常水平。然而,在完全缓解仅3个月后,患者对巩固化疗产生耐药,并出现骨髓复发迹象。尽管我们增加了药物剂量并尝试使用不同的方案,但她在首次入院近10个月后死于严重贫血和出血。总之,前体B细胞ALL是以肾浸润为首发体征的最常见白血病类型。由于ALL的预后较差,一些新的治疗方法可能会改善患者的病情。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/982b/5642469/0d0bd61c3685/CNCS-5-060-01.jpg

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