Yoshimoto Koji, Maeoka Yujiro, Kubota Shiori, Chishaki Ren, Takahashi Akira, Osaki Yosuke, Mino Tatsuji, Ichinohe Tatsuo, Masaki Takao
Department of Nephrology, Hiroshima University Hospital, 1-2-3 Kasumi, Minami-ku, Hiroshima, 734-8551, Japan.
Department of Hematology and Oncology, Research Institute for Radiation Biology and Medicine, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima, 734-8553, Japan.
BMC Nephrol. 2025 Mar 6;26(1):121. doi: 10.1186/s12882-025-04032-3.
Polycythemia is a rare disease that can cause hypertension. Secondary polycythemia with increased production of erythropoietin (EPO) is associated with several kidney diseases, including hydronephrosis and cystic disease. However, there have been no reports of a case presenting with polycythemia secondary to bilateral nephromegaly caused by renal infiltration of T-cell acute lymphoblastic leukemia (T-ALL).
A 32-year-old Japanese man presented with marked hypertension (215/150 mmHg) with renal insufficiency (creatinine 3.7 mg/dL), proteinuria, hematuria, bilateral nephromegaly, polycythemia (hemoglobin 20.2 g/dL), and increased serum EPO (38.7 mIU/mL, range 4.2-23.7 mIU/mL). Based on renal and bone marrow biopsy findings, he was diagnosed with T-ALL and bilaterally enlarged kidneys caused by renal infiltration of leukemic cells. There was no evidence of endocrine hypertension or fluid retention. Remission induction chemotherapy led to a decrease in kidney size, hemoglobin levels, and serum EPO levels, and allowed dose reductions of most hypertensive drugs, suggesting that hypertension was secondary to polycythemia. The patient's renal function gradually improved and hemodialysis was discontinued after 1 month of chemotherapy.
We report a case of marked hypertension and secondary polycythemia induced by severe renal infiltration of T-ALL at diagnosis, which were synchronically improved with induction chemotherapy. This case history suggests the importance of considering lymphoproliferative diseases in the differential diagnosis of secondary polycythemia, leading to severe hypertension.
真性红细胞增多症是一种可导致高血压的罕见疾病。促红细胞生成素(EPO)生成增加所致的继发性真性红细胞增多症与包括肾积水和囊性疾病在内的多种肾脏疾病相关。然而,尚无因T细胞急性淋巴细胞白血病(T-ALL)肾脏浸润导致双侧肾肿大继发真性红细胞增多症的病例报告。
一名32岁日本男性,患有严重高血压(215/150 mmHg),伴有肾功能不全(肌酐3.7 mg/dL)、蛋白尿、血尿、双侧肾肿大、真性红细胞增多症(血红蛋白20.2 g/dL)以及血清EPO升高(38.7 mIU/mL,范围4.2 - 23.7 mIU/mL)。根据肾脏和骨髓活检结果,他被诊断为T-ALL以及白血病细胞肾脏浸润导致的双侧肾脏肿大。无内分泌性高血压或液体潴留的证据。诱导缓解化疗使肾脏大小、血红蛋白水平和血清EPO水平降低,并允许减少大多数降压药物的剂量,提示高血压继发于真性红细胞增多症。化疗1个月后患者肾功能逐渐改善,血液透析停止。
我们报告一例诊断时因T-ALL严重肾脏浸润导致显著高血压和继发性真性红细胞增多症的病例,诱导化疗使其同步改善。该病例史提示在继发性真性红细胞增多症导致严重高血压的鉴别诊断中考虑淋巴增殖性疾病的重要性。