Silva Gyl Eb, Teixeira André C, Vergna José Gg, Salgado-Filho Natalino, Crivellentti Leandro Z, Costa Roberto S, Dantas Márcio
Department of Pathology, Ribeirão Preto Medical School, University of São Paulo Brazil.
Division of Nephrology, Ribeirão Preto Medical School, University of São Paulo Brazil.
Int J Clin Exp Med. 2014 Jun 15;7(6):1619-22. eCollection 2014.
Sickle cell disease is a severe disease with a genetic pattern; it may cause anemia, vaso-occlusive phenomena, and multiorgan injury. It may damage any renal compartment, thereby causing tubular abnormalities, papillary necrosis, or glomerulopathies such as focal and segmental glomerulosclerosis and membranoproliferative pattern. The clinical consequences are hematuria and proteinuria. Hematuria associated with SCD is characteristically isomorphic (non-glomerular). This case report describes a novel case of a patient with sickle cell disease who presented with proteinuria and microscopic dysmorphic (glomerular) hematuria. A renal biopsy revealed immunoglobulin A nephropathy. Despite the fact that immunoglobulin A nephropathy is the most commonly diagnosed glomerulonephritis worldwide, an association between this entity and sickle cell disease has not yet been reported, probably because all cases of hematuria in patients with sickle cell disease have been regarded as secondary to sickle cell disease. Thus, new approaches are necessary to differentiate these conditions, such as evaluation of urinary erythrocyte dysmorphism, even more so because these two entities have different therapeutic options, morbidity, and mortality rates.
镰状细胞病是一种具有遗传模式的严重疾病；它可能导致贫血、血管阻塞现象和多器官损伤。它可能损害肾脏的任何部分，从而导致肾小管异常、乳头坏死或肾小球病变，如局灶节段性肾小球硬化和膜增生性病变。临床后果是血尿和蛋白尿。与镰状细胞病相关的血尿典型地为均一性（非肾小球性）。本病例报告描述了一例镰状细胞病患者出现蛋白尿和镜下异形（肾小球性）血尿的新病例。肾活检显示为免疫球蛋白A肾病。尽管免疫球蛋白A肾病是全球最常诊断的肾小球肾炎，但该疾病实体与镰状细胞病之间的关联尚未见报道，可能是因为镰状细胞病患者的所有血尿病例都被认为是继发于镰状细胞病。因此，需要新的方法来区分这些情况，如评估尿红细胞异形性，尤其是因为这两种疾病实体有不同的治疗选择、发病率和死亡率。
