Sungur C, Sungur A, Ruacan S, Arik N, Yasavul U, Turgan C, Caglar S
Department of Nephrology, Hacettepe University, School of Medicine, Ankara, Turkey.
Kidney Int. 1993 Oct;44(4):834-6. doi: 10.1038/ki.1993.318.
Systemic AA type amyloidosis with renal involvement is the major cause of morbidity and mortality in patients with familial Mediterranean fever (FMF). A histopathological examination is essential to achieve a definite diagnosis in systemic amyloidosis. The diagnostic yield of the procedure varies according to the biopsy site and renal biopsy has the highest yield. On the other hand this procedure has its own complications and requires hospitalization of the patient. Alternative biopsy sites have been proposed with varying degrees of sensitivity and morbidity to reduce the morbidity and mortality of solid organ biopsies. We performed bone marrow biopsies in 39 patients with FMF who had different stages of renal disease. Thirty-one (79.5%) of the 39 specimens showed significant perivascular amyloid infiltration when stained with crystal violet and Congo red. An immunoperoxidase stain with a monoclonal antibody proved that these deposits were AA type amyloid. We suggest that bone marrow biopsy can be utilized for a safe and quick diagnosis of systemic amyloidosis in patients with FMF and renal disease.
伴有肾脏受累的系统性AA型淀粉样变性是家族性地中海热（FMF）患者发病和死亡的主要原因。组织病理学检查对于确诊系统性淀粉样变性至关重要。该检查的诊断成功率因活检部位而异，肾活检的成功率最高。另一方面，该检查有其自身的并发症，且需要患者住院。已提出了不同敏感性和发病率的替代活检部位，以降低实体器官活检的发病率和死亡率。我们对39例处于不同肾病阶段的FMF患者进行了骨髓活检。39份标本中有31份（79.5%）经结晶紫和刚果红染色后显示有明显的血管周围淀粉样浸润。用单克隆抗体进行的免疫过氧化物酶染色证明这些沉积物为AA型淀粉样蛋白。我们建议骨髓活检可用于安全、快速诊断患有FMF和肾病的患者的系统性淀粉样变性。
