Ray S, Pavithran K, Nair P S
Internal Medicine, Amrita Institute of Medical Sciences and Research Centre, Elamakkara, Kerala.
J Assoc Physicians India. 2006 Jul;54:577-80.
Bone involvement in primary systemic amyloidosis is rare. Intracranial involvement in primary amyloidosis has not been reported so far. We report two cases of bone involvement in primary amyloidosis. The first patient also had combined deficiencies of factor IX and XII, while the second patient had associated intracranial involvement and XIIth cranial nerve palsy. Both these cases are unique in that, destructive bone lesions with intracranial involvement and combined factor deficiencies have not been reported in primary amyloidosis previously.
原发性系统性淀粉样变性累及骨骼较为罕见。原发性淀粉样变性累及颅内目前尚未见报道。我们报告两例原发性淀粉样变性累及骨骼的病例。首例患者还合并因子IX和XII缺乏,而第二例患者伴有颅内受累及第十二对脑神经麻痹。这两例病例均很独特,因为原发性淀粉样变性此前未曾报道过伴有颅内受累的破坏性骨病变及合并因子缺乏的情况。