Muhammad Khan Muzammil, Ur Rashid Mamoon, Ullah Waqas, Hussain Ishtiaq, Hurairah Abu
Internal Medicine, Khyber Medical College, Peshawar, Pakistan.
Internal Medicine, AdventHealth Dade City, Dade City, Florida, USA.
BMJ Case Rep. 2020 Jan 28;13(1):e231563. doi: 10.1136/bcr-2019-231563.
Amyloidosis is the extracellular deposition of unique protein fibrils in different tissue organs. It is most commonly associated with B-cell malignancy such as multiple myeloma or Waldenstrom macroglobulinaemia. It involves the liver, heart, kidney, peripheral nerves and soft tissues. Liver however is affected, but clinically apparent disease is very rare. Hepatomegaly and mild elevation of alkaline phosphatase is the most common presentation in patients with liver involvement. Acute hepatic failure is a rare presentation with myeloma-induced amyloidosis. The diagnosis can be difficult requiring biopsy or sometimes special staining of the tissue. Management is still very challenging.
淀粉样变性是独特的蛋白质原纤维在不同组织器官中的细胞外沉积。它最常与B细胞恶性肿瘤相关，如多发性骨髓瘤或华氏巨球蛋白血症。它累及肝脏、心脏、肾脏、周围神经和软组织。肝脏虽会受到影响，但临床上明显的疾病非常罕见。肝肿大和碱性磷酸酶轻度升高是肝脏受累患者最常见的表现。急性肝衰竭是骨髓瘤诱导的淀粉样变性的罕见表现。诊断可能困难，需要进行活检，有时还需要对组织进行特殊染色。治疗仍然非常具有挑战性。
