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急性淋巴细胞白血病(ALL)的孤立性中枢神经系统复发:来自三级医疗中心的经验。

Isolated CNS Relapse in Acute Lymphoblastic Leukemia (ALL): An Experience from a Tertiary Care Center.

作者信息

Tevatia Manvir Singh, Sharma Isha, Jadhav Toyaja, Somasundaram Venkatesan, Sharma Sanjeevan

机构信息

Department of Pathology, Command Hospital, Pune, Maharashtra, India.

Department of Pathology, Armed Forces Medical College, Pune, Maharashtra, India.

出版信息

J Lab Physicians. 2021 Jun;13(2):134-138. doi: 10.1055/s-0041-1730752. Epub 2021 Jun 15.

Abstract

In this study the various parameters of acute lymphoblastic leukemia (ALL), including the clinical features, peripheral blood and bone marrow (BM) findings, immunophenotypic and cytogenetic details in ALL cases who had isolated relapse involving the central nervous system (CNS), were studied.  Duration of the study is from 2015 to 2019 in which 5 ALL cases were presented to this tertiary care center. The presenting symptoms varied from headache, fever, and distension of abdomen. These cases were either on therapy or post completion of chemotherapy. The diagnosis of CNS relapse followed after the examination of cerebrospinal fluid (CSF). Patients also underwent BM examination to rule out systemic relapse.  Age of patients ranged from 7 months to 42 years. There were three female patients. Two patients had isolated CNS relapse 3.5 years after completing therapy and succumbed to their illness. Two patients had t(9;22) while one patient had t(1;14) cytogenetic abnormality at diagnosis. One patient was diagnosed as T-ALL. Treatment offered was German Multicentre ALL protocol for induction along with 10 cycles of maintenance.  The most common hematolymphoid malignancy in children namely ALL accounts for 75% of childhood leukemias. Complete remission rates reach up to 70 to 80%. CNS involvement is known to occur in these cases. CNS relapse may occur alone or with systemic relapse. Advances in therapeutic protocols along with CNS prophylaxis have drastically brought down the rates of CNS relapse. It is essential to maintain a high degree of suspicion so that these cases of isolated CNS relapse can be identified at the earliest and definitive therapy can be offered.

摘要

在本研究中,对急性淋巴细胞白血病(ALL)的各种参数进行了研究,包括临床特征、外周血和骨髓(BM)检查结果、孤立性复发累及中枢神经系统(CNS)的ALL病例的免疫表型和细胞遗传学细节。本研究为期2015年至2019年,期间有5例ALL病例被送至该三级护理中心。呈现的症状包括头痛、发热和腹部胀满。这些病例要么正在接受治疗,要么化疗已结束。脑脊液(CSF)检查后确诊为CNS复发。患者还接受了BM检查以排除全身复发。患者年龄从7个月至42岁不等。有3名女性患者。2例患者在完成治疗3.5年后出现孤立性CNS复发并因病死亡。2例患者在诊断时存在t(9;22),1例患者存在t(1;14)细胞遗传学异常。1例患者被诊断为T-ALL。给予的治疗方案是德国多中心ALL诱导方案及10个周期的维持治疗。儿童中最常见的血液淋巴系统恶性肿瘤即ALL占儿童白血病的75%。完全缓解率可达70%至80%。已知这些病例会发生CNS受累。CNS复发可能单独发生或与全身复发同时发生。治疗方案的进展以及CNS预防措施已大幅降低了CNS复发率。必须保持高度怀疑,以便尽早识别这些孤立性CNS复发病例并提供确定性治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5b2f/8409115/6fa5fee8d22d/10-1055-s-0041-1730752_7_0268_01.jpg

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