Department of Hematology, Teine Keijinkai Medical Center, Sapporo, Hokkaido, Japan.
Department of Nephrology, Teine Keijinkai Medical Center, Sapporo, Hokkaido, Japan.
CEN Case Rep. 2023 Feb;12(1):56-62. doi: 10.1007/s13730-022-00721-2. Epub 2022 Jul 20.
Crystalline light chain cast nephropathy is a rare distinct morphologic variant of light chain cast nephropathy which is the most common renal lesion associated with multiple myeloma. It is often related to high myeloma tumor burden, severe acute kidney injury, and an unfavorable prognosis. A 79-year-old Japanese man was referred to our medical center with anemia, proteinuria, and acute exacerbation of the serum creatinine accompanying anuria. A renal biopsy showed crystalline cast filling the tubular lumens, injured tubular cells, and inflammatory cells infiltration of interstitium. Serum and urine immunofixation detected a monoclonal protein (IgA-λ and Bence-Jones Protein-λ, respectively), and bone marrow examination observed 64% of plasma cells. IgA-λ type multiple myeloma-associated crystalline light chain cast nephropathy and accompanying acute kidney injury were confirmed. Hydration and emergency hemodialysis were immediately introduced, and the treatment with bortezomib and dexamethasone was initiated. The patient showed successful recovery in renal manifestations. We suggest that early use with bortezomib-based therapy should be considered for patients with acute kidney injury caused by multiple myeloma-associated crystalline light chain cast nephropathy.
结晶型轻链管型肾病是轻链管型肾病的一种罕见的独特形态学变异，是多发性骨髓瘤最常见的与肾脏相关的病变。它常与高骨髓瘤肿瘤负担、严重急性肾损伤和不良预后有关。一名 79 岁的日本男性因贫血、蛋白尿和伴有无尿的血清肌酐急性恶化而被转至我们的医疗中心。肾活检显示结晶管型填充在管状腔中，损伤的管状细胞和间质中有炎症细胞浸润。血清和尿液免疫固定电泳检测到单克隆蛋白（分别为 IgA-λ 和 Bence-Jones 蛋白-λ），骨髓检查发现 64%的浆细胞。确诊为 IgA-λ 型多发性骨髓瘤相关结晶型轻链管型肾病伴急性肾损伤。立即进行补液和紧急血液透析，并开始使用硼替佐米和地塞米松治疗。该患者的肾脏表现成功恢复。我们建议，对于由多发性骨髓瘤相关结晶型轻链管型肾病引起的急性肾损伤患者，应考虑早期使用硼替佐米为基础的治疗。
