无明显内脏损害的原发性系统性淀粉样变病2例

报告2例原发性系统性淀粉样变病患者,均表现为皮肤瘀斑,皮肤组织病理也可见大片的淀粉样物质在血管壁上的沉积。两例患者均伴有血清IgA和尿κ轻链升高,但尚未发现明显的内脏受累。患者1免疫电泳有单克隆条带,骨髓浆细胞增生,符合骨髓瘤诊断标准;患者2缺乏免疫产物单克隆增生的证据,骨髓无明显浆细胞增生,只能诊断浆细胞失调,但其疾病未来的转归尚有待长期观察。

Two Cases of Primary Systemic Amyloidosis without Obvious Visceral Involvement

To report two cases of primary systemic amyloidosis with ecchymoses and deposition of amyloid substances on dermal vessel walls histologically. Serum IgA and urine κ light chain were elevated in both cases,but no obvious visceral involvement were found neither. Monoclonal band was found by immunoelectrophoresis of the first patient and the plasma cell proliferation in the bone marrow suggested the diagnosis of myeloma. The second patient was diagnosed as plasma cell dyscrasia due to absence of the above clues,and has been followed up after discharge.