急性淋巴细胞白血病是最常见的儿科恶性肿瘤，特点是发烧、贫血、出血以及原始细胞浸润器官引起的症状。这是一名 9 岁亚洲急性淋巴细胞白血病患者的病例报告，患者仅表现为多尿作为没有任何其他临床表现的表现特征； 高度怀疑原发性肾脏疾病或遗传性代谢疾病。但禁水试验和禁水加压试验提示肾性尿崩症，肾活检显示肾间质弥漫性淋巴细胞浸润。立即进行骨髓穿刺，最终综合诊断为B淋巴细胞白血病。白血病母细胞肾浸润大多无症状，但我们的病例提示可表现为肾性尿崩症。本病例充分说明急性淋巴细胞白血病髓外浸润的表现是多种多样的。当患者以肾性尿崩症为首发症状时，查找病因时应考虑血液肿瘤浸润的可能，并及时进行骨髓细胞学检查。© 2024. 作者。

Acute lymphoblastic leukemia is the most common pediatric malignancy, characterized by fever, anemia, hemorrhage, and symptoms brought on by blasts infiltrating organs.This is a case report of a 9-year-old Asian patient with acute lymphoblastic leukemia who presented with polyuria alone as a presenting feature without any other clinical manifestation; primary renal disease or inherited metabolic disease was highly suspected. However, the water deprivation test and water deprivation pressurization test suggested nephrogenic diabetes insipidus, and the renal biopsy displayed diffuse lymphocytic infiltration in the renal interstitium. Bone marrow aspiration was performed immediately, and a comprehensive diagnosis of B-lymphoblastic leukemia was finally made.Renal infiltration with leukemic blasts mostly remains asymptomatic, but our case suggests that it can present with nephrogenic diabetes insipidus. This case fully demonstrates that the presentation of extramedullary infiltration in acute lymphoblastic leukemia is varied. When the patient has renal diabetes insipidus as the first symptom, the possibility of hematological tumor infiltration should be considered when finding the cause, and timely bone marrow cytology should be performed.© 2024. The Author(s).