Schwarzinger I, Stain-Kos M, Bettelheim P, Pabinger I, Kyrle P, Kalhs P, Kapiotis S, Jäger U, Lechner K
1st Medical Department, University of Vienna, Austria.
Thromb Haemost. 1992 Dec 7;68(6):648-51.
We describe the case of a 64-year-old woman with isolated severe factor X deficiency associated with kappa light chain myeloma. At the time of diagnosis there was no evidence for amyloidosis. Complete remission (CR) of myeloma as well as normalization of factor X levels were achieved after cytostatic chemotherapy. Subsequently, factor X deficiency recurred twice without any evidence for relapse of myeloma. The first time factor X normalized again following cytostatic treatment, the second time, however, factor X deficiency was refractory to chemotherapy. Finally, relapse of myeloma became evident associated with rapidly progressing, systemic amyloidosis, which was fatal within a few months. Initially, factor X infusion studies showed a normal recovery, but when amyloidosis became overt the recovery decreased to 0%. We assume that factor X deficiency was due to a binding of factor X to kappa light chains associated with the proliferation of the malignant myeloma cell clone.
我们描述了一例64岁女性患者，其患有与κ轻链骨髓瘤相关的孤立性严重因子X缺乏症。诊断时没有淀粉样变性的证据。细胞抑制化疗后实现了骨髓瘤的完全缓解（CR）以及因子X水平的正常化。随后，因子X缺乏症复发了两次，且没有任何骨髓瘤复发的证据。第一次细胞抑制治疗后因子X再次恢复正常，然而，第二次时，因子X缺乏症对化疗无效。最后，骨髓瘤复发并伴有迅速进展的全身性淀粉样变性，患者在几个月内死亡。最初，因子X输注研究显示恢复正常，但当淀粉样变性明显时，恢复率降至0%。我们推测因子X缺乏是由于因子X与与恶性骨髓瘤细胞克隆增殖相关的κ轻链结合所致。
