Morschhauser F, Wattel E, Pagniez D, Lovi V, Rose C, Bauters F, Fenaux P
Service des Maladies du Sang, CHU Lille, France.
Leuk Lymphoma. 1995 Aug;18(5-6):479-83. doi: 10.3109/10428199509059648.
Among 825 cases of de novo myelodysplastic syndromes (MDS) diagnosed over a period of 13 years in our center, 4 had clinically significant glomerulopathy. All 4 fulfilled diagnostic criteria of chronic myelomonocytic leukemia (CMML), and could be classified in the low or intermediate risk groups according to two scoring systems. Presenting symptoms of renal involvement were edema in 3 cases and acute renal failure in the remaining patient. Three patients had the nephrotic syndrome. Renal biopsy (performed in 2 cases but considered as contraindicated in the other cases) showed AL amyloidosis on one case and extracapillary glomerulonephritis in the other case. The 4 patients were treated with V16 or hydroxyurea and two had renal improvement. Only one previous case of MDS associated with glomerulopathy has been reported before and also very probably had CMML. This, and the response of renal disease to chemotherapy in 2 of our patients suggests a possible relationship between the two disorders. More systematic investigation of glomerular function, in CMML, could possibly disclose a higher incidence of cases of glomerular injury in this type of MDS.
在我们中心13年间诊断的825例初诊骨髓增生异常综合征(MDS)患者中,有4例出现具有临床意义的肾小球病变。这4例均符合慢性粒单核细胞白血病(CMML)的诊断标准,并且根据两种评分系统可归类为低危或中危组。肾脏受累的表现症状为3例出现水肿,其余1例为急性肾衰竭。3例患者患有肾病综合征。肾活检(2例进行了活检,另2例认为不适合进行活检)显示,1例为AL淀粉样变性,另1例为毛细血管外肾小球肾炎。这4例患者接受了V16或羟基脲治疗,其中2例肾脏情况有所改善。此前仅报道过1例与肾小球病变相关的MDS病例,该病例很可能也患有CMML。我们的2例患者中肾脏疾病对化疗有反应,这表明这两种疾病之间可能存在关联。对CMML患者的肾小球功能进行更系统的研究,可能会发现这类MDS中肾小球损伤病例的发生率更高。