Lafargue Marie-Camille, Bobot Mickaël, Rennke Helmut G, Essig Marie, Carre Martin, Mercadal Lucile, Farhi Jonathan, Sakhi Hamza, Comont Thibault, Golbin Léonard, Isnard Pierre, Chemouny Jonathan, Cambier Nathalie, Laribi Kamel, Selamet Umut, Riella Leonardo V, Fain Olivier, Adès Lionel, Fenaux Pierre, Cohen Camille, Mekinian Arsène
Department of Nephrology, Tenon's Hospital, Assistance Publique-Hôpitaux de Paris, Université Paris Cité, Paris, France.
Centre de Néphrologie et Transplantation Rénale, AP-HM, Hôpital de la Conception, CHU de la Conception, 13005, Marseille, France.
Kidney Int Rep. 2023 Sep 7;8(12):2733-2741. doi: 10.1016/j.ekir.2023.09.005. eCollection 2023 Dec.
Chronic myelomonocytic leukemia (CMML) is a hematologic disorder that is an overlap syndrome between myelodysplastic syndromes and myeloproliferative neoplasms, and can be associated with autoimmune and inflammatory diseases. This study aimed to describe kidney involvement in patients with CMML, their treatments, and outcomes.
We conducted a French and American multicenter retrospective study in 15 centers, identifying patients with CMML with acute kidney injury (AKI), chronic kidney disease (CKD), and urine abnormalities.
Sixteen patients (males, = 14; median age 76.5 years [71.9-83]) developed a kidney disease 6 months [1.6-25.6] after the diagnosis of CMML. At the time of kidney disease diagnosis, median urinary protein-to-creatinine ratio was 2 g/g [1.25-3.4], and median serum creatinine was 2.26 mg/dl [1.46-2.68]. Fourteen patients (87.5%) underwent a kidney biopsy, and the 2 main pathological findings were lysozyme nephropathy (56%) and renal infiltration by the CMML (37.5%). Ten patients received a new treatment following the CMML-associated kidney injury. Among patients with monitored kidney function, and after a median follow-up of 15 months [9.9-34.9], 4 patients had CKD stage 3, 4 had CKD stage 4, 1 had an end-stage kidney disease. In our patient series, 2 patients evolved to an acute myeloid leukemia (AML), and 5 died. Compared with 116 CMML controls, patients who had a kidney involvement had a higher monocyte count ( < 0.001), had more CMML-1 ( = 0.005), were more susceptible to develop an AML ( = 0.02), and were more eligible to receive a specific hematologic treatment, with hydroxyurea, or hypomethylating agents ( < 0.001), but no survival difference was seen between the 2 groups ( = 0.6978).
In this cohort of patients with CMML with a kidney injury, the 2 most frequent renal complications were lysozyme-induced nephropathy and renal infiltration by the CMML. Kidney involvement should be closely monitored in patients with CMML.
慢性粒单核细胞白血病（CMML）是一种血液系统疾病，是骨髓增生异常综合征和骨髓增殖性肿瘤之间的重叠综合征，可与自身免疫性和炎性疾病相关。本研究旨在描述CMML患者的肾脏受累情况、治疗方法及预后。
我们在15个中心开展了一项法国和美国多中心回顾性研究，纳入患有急性肾损伤（AKI）、慢性肾脏病（CKD）及尿液异常的CMML患者。
16例患者（男性14例；中位年龄76.5岁[71.9 - 83岁]）在CMML诊断后6个月[1.6 - 25.6个月]出现肾脏疾病。在肾脏疾病诊断时，尿蛋白肌酐比值中位数为2 g/g[1.25 - 3.4]，血清肌酐中位数为2.26 mg/dl[1.46 - 2.68]。14例患者（87.5%）接受了肾脏活检，2个主要病理表现为溶菌酶肾病（56%）和CMML肾浸润（37.5%）。10例患者在CMML相关肾损伤后接受了新的治疗。在肾功能监测的患者中，中位随访15个月[9.9 - 34.9个月]后，4例患者为CKD 3期，4例为CKD 4期，1例为终末期肾病。在我们的患者系列中，2例患者进展为急性髓系白血病（AML），5例死亡。与116例CMML对照相比，有肾脏受累的患者单核细胞计数更高（P < 0.001），CMML-1更多（P = 0.005），更易发生AML（P = 0.02），更适合接受特定的血液学治疗，如羟基脲或去甲基化药物（P < 0.001），但两组间生存率无差异（P = 0.6978）。
在这组有肾损伤的CMML患者中，2种最常见的肾脏并发症为溶菌酶诱导的肾病和CMML肾浸润。CMML患者的肾脏受累情况应密切监测。
